Keywords in Dermatology and Venereology

FRCP

2012-02-07T21:06:00.004+02:00

I have been elevated to the Fellowship status, FRCP (Fellow of the Royal College of Physicians), Glasgow. The Royal College was established in 1599.

Quiz

2012-02-04T00:41:00.004+02:00

Figure 1

Figure 2

There are several modes of ultrasonography among which is the duplex (Latin, two) ultrasonography which combines conventional ultrasonography with Doppler (named after physicist C Doppler) ultrasonography. Ultrasonography creates pictures while Doppler ultrasonography measures and visualises blood flow.

Pharmaco-penile duplex ultrasonography (PPDU) is duplex ultrasonography with intracavernosal injection of vasoactive substance(s) where, the penile arteries are examined by a combination of conventional ultrasound and a Doppler ultrasonography to measure velocity.

What do the findings in figure 1 suggest?

What do the findings in figure 2 suggest?
Is direct measurement of velocity in deep dorsal vein of the penis useful in PPDU?

Does oral sildenafil have a role in PPDU?

The answers will be provided below as comments.

This page was last updated in February 2012.

Quiz

2012-01-31T02:44:00.005+02:00

Testicular size is determined by palpation and comparison to testis-shaped models of defined sizes (Prader orchidometer).

What is the most reliable sign for the onset of puberty in males?

What is the clinical significance of megalotestis (macroorchidism)?

Te answers are provided below as comments.

This page was last updated in February 2012.

Diseases that may lead to a leonine facies

2012-01-29T18:19:00.007+02:00

Diseases that may lead to a leonine facies

Carcinoid syndrome
Chronic actinic dermatitis/actinic reticuloid
Focal facial dermal dysplasia
KID syndrome (keratitis, ichthyosis, deafness)
Leishmaniasis
Lepromatous Leprosy
Lipoid proteinosis
Lymphoma, leukaemia
Mycosis fungoides
Multicentric reticulohistiocytosis
Multiple keratoacanthoma syndrome
Pachydermoperiostosis/cutis verticis gyrata
Paget's disease of bone (the patient may first complain that hats no longer fit properly due to cranial enlargement)
Progressive nodular histiocytosis
Pseudophotodermatitis (when chronic, airborne pattern of allergic contact dermatitis induced by Compositae plants)
Rosacea
Sarcoidosis
Scleromyxoedema

Primary pachydermoperiostosis

This page was last updated in January 2012.

Quiz

2012-01-24T21:53:00.011+02:00

This man who has sex with men had no history of similar attacks of this painful condition in the anal area before. What is the most likely diagnosis? How to confirm it? Does a concomitant HIV infection affect your management? If it does, explain how? Standard STI screening has ensued.

Short and long answers are provided below as comments.

This page was last updated in February 2012.

Types of halo naevi

2012-01-24T07:34:00.014+02:00

Type of halo naevus (5 types)	Features
1. Classic halo naevus with histopathologically apparent inflammation (the infiltrate contains many cytotoxic T-cells, and may represent immunologically-induced rejection).	The classic lesion is a brown naevus with a surrounding halo of vitiligo-like depigmentation. The naevus usually undergoes involution (losing its pigment then disappears) and the halo usually repigments. In unusual lesions, darkening of the naevus rather than lightening has been described (? postinflammatory hyperpigmentation).
2. Halo naevus with no histopathologically apparent inflammation.	In such instances, the naevus does not undergo involution.
3. Halo naevus without halo	It is actually without a clinical halo but with histopathologically apparent inflammation. The naevus may or may not undergo involution.
4. Inverse halo naevus^*	It is actually a centrifugal dissolving naevus (a centrifugal destruction of melanocytes producing clinically an annular naevoid ring).
5. Pseudo-halo naevus	It is merely produced by the application of sunscreen to a naevus and its immediate surround.

Circulating antibodies that react against melanoma cells have been found in a high proportion of individuals with halo naevi, as have circulating lymphocytes with an activated phenotype, suggesting that both humoral and cell-mediated immunity are involved in the rejection of naevus cells and the formation of the halo. Interestingly, fibrosis does not occur in the dermis as a consequence of the halo naevus regression, whereas it does occur in regressing melanomas. This difference may result from the higher expression of the antifibrotic cytokine tumour necrosis factor-ɑ (TNF-ɑ) in halo naevi than in regressing melanomas.

A full mucocutaneous examination at the time of diagnosis is indicated to exclude a concurrent melanoma. The decision to remove the naevus at the centre of the halo is based on its morphologic features, just as with any other naevus. Not infrequently, halo naevi are multiple, occurring either simultaneously or successively. There may be associated vitiligo and/or Turner’s syndrome. Note that halo dermatitis around a melanocytic naevus is called Meyerson’s naevus and it usually resolves spontaneously within a few months, without involution of the naevus. This differs from halo naevus (also called Sutton’s naevus), although the two conditions have been reported to coexist in the same patient, and progression to Sutton’s naevus has occurred.

* Nashan D, Meiss F, Braun-Falco M, Hofbauer M, Hofmann S. Multiple target-like pigmented nevi: an inverse halo-nevus phenomenon. J Eur Acad Dermatol Venereol 2010; 24: 104–105.

This page was last updated in January 2012.

Electrodesiccation of an acrochordon

2012-01-24T04:58:00.007+02:00

Acrochordon = skin tag = fibroepithelial polyp

Desiccation = dehydration

Non-sterile disposable gloves are adequate for procedures like curettage and shave biopsies. Sterile gloves are used e.g. in suture surgery.

Haemostasis can only be achieved satisfactorily if the area being diathermied is relatively dry.

Monoterminal procedures (without a dispersive plate) produce desiccation/fulguration.

This page was last updated in January 2012.

Immune restoration syndrome

2012-01-12T23:15:00.005+02:00

Annual ESDV Conference 2012
Marriott Hotel, Zamalek, Cairo, Egypt

Immune restoration syndrome

Wednesday 18/1/2012 at 2:45 pm

Abstract: Immune restoration syndrome, also called immune reconstitution (inflammatory) syndrome (IRIS), has two clinical scenarios: unmasking and paradoxical. This will be discussed in relation to HIV infection. Immune restoration Klebsiella lymphadenitis has been reported for the first time few months ago and will be described.

http://www.4shared.com/office/YdwnR036/IRIS_2012.html

Relevant paper:

Immune restoration Klebsiella lymphadenitis

Gohar A. Immune restoration Klebsiella lymphadenitis. International J STD AIDS 2011; 22:536-7.

Immune restoration syndrome unmasking Klebsiella lymphadenitis was reported in an HIV infected patient for the first time.

This page was last updated in January 2012.

Quiz

2011-11-05T07:45:00.006+02:00

12-year old patient presented with this type of scaling [scales, large “plate-like” , very thick, dark brown and firmly adherent, have been over most of the skin surface including the face (A and B) and accentuated on lower extremities (C)] which started within the first month of life. Which congenital disorder of cornification is this?

Normally, cornification involves the construction of the cornified envelope from intracellular protein precursors including loricrin and involucrin. These are cross-linked to lipid ceramides in the granular layer by the action of transglutaminase 1. Kinetic studies in this disease revealed a near-normal transit time, and it is therefore classified as a retention disorder of cornification (retention hyperkeratosis with hypergranulosis). Mutations in the keratinocyte transglutaminase-1 gene on chromosome 14q11.1 interfere with the crosslinkage of loricrin and involucrin and the formation of the cornified cell envelope (retention hyperkeratosis). What are the other congenital disorders of cornification that show retention hyperkeratosis?

Short and long answers are provided below as comments.

Quiz

2011-11-03T23:30:00.003+02:00

The history is characteristic. This 22-year old patient with a common fungal infection was satisfied initially with the cream prescribed by another doctor. He stopped applying the cream, the eruption relapsed. Further applications brought renewed relief and the cycles were repeated. He developed few persistent nodules, which become insuppressible by the cream. Scaling was lost in the groins areas as shown (A), and bruise-like brownish discolouration was seen (A). Scaling (arrows) was still seen on the buttocks (B). From which site autoinfection can occur?

Short and long answers are provided below as comments.

Electrosurgical waveforms

2011-10-31T20:10:00.003+02:00

Electrosurgery (diathermy) equipment converts domestic alternating current into high-frequency alternating current (of various types) which is converted to heat energy as it passes THROUGH a high-resistance medium such as the skin. Haemostasis can only be achieved satisfactorily if the area being diathermied is relatively dry. Each type of electrosurgical current produces its own wavy pattern of current flow, called the waveform.

Different electrical waveforms produce tissue desiccation/fulguration, coagulation, or cutting. Some machines are designed to produce all effects. There is however some overlap between these effects so that the desiccation waveform (dehydration) can be used, albeit not perfectly, for coagulation and cutting.

Monoterminal procedures (without a dispersive plate) produce desiccation/fulguration. Biterminal procedures produce coagulation with a dispersive plate or bipolar electrode (bipolar forceps, keeps the current flow on the surface travelling from one tine of the forceps to the other) or produce coagulation with cutting or pure cutting without appreciable coagulation effect (electrosection) with a dispersive plate. Pure cutting is useful in diagnostic biopsy avoiding the tissue damage associated with coagulation (after the biopsy is taken, haemostasis can be attained by switching to coagulation).

In electrofulguration, the needle tip is not in contact with the skin. As the spark jumps between the skin and the needle its energy is spread over a greater area, resulting in more superficial tissue damage (superficial dehydration). Deep penetration does not occur because the charring acts as an insulating barrier.

Quiz

2011-10-27T06:58:00.006+02:00

What does the development of an exophytic nodule on this naevus in the child patient, that has been present since birth, usually represent? Does the patched-hedgehog signalling pathway malfunction in this regard? What advice would you give the parent?

In this naevus, around puberty there is enlargement of the

sebaceous glands, which are often located abnormally high in the

dermis, with an increased number of closely set lobules and

malformed ducts (A). A variety of appendageal tumours, sometimes

multiple, may develop within sebaceous naevi. The most commonly

reported are trichoblastoma and syringocystadenoma papilliferum

(B). It should be noted that basaloid proliferations (arrow)

that are seen in as many as half of all cases of naevus sebaceous

are not always easily differentiated from basal cell carcinoma

(BCC). Misinterpretation of trichoblastoma and basaloid

proliferation as BCC has caused problems in the past.

How to differentiate between BCC and trichoblastoma

pathologically?

Short and long answers are provided below as comments.

Quiz

2011-10-25T07:11:00.007+02:00

Non-Langerhans cell histiocytosis represents a broad group of different disorders characterized by the proliferation of histiocytes other than Langerhans cells. One of which, is multicentric reticulohistiocytosis shown here in this middle-aged woman with yellow-pink papules and nodules (with scleral involvement). Multicentric reticulohistiocytosis has subtype (multiple reticulohistiocytomas) that is restricted to skin. It is possible that the diffuse, purely cutaneous form is an early stage of multicentric reticulohistiocytosis before the appearance of joint and other lesions. In multicentric reticulohistiocytosis the onset of arthropathy, may precede, follow, or accompany the onset of skin lesions. No treatment is of consistent value for this disease.

The characteristic pathological picture in multicentric reticulohistiocytosis is of infiltration by mononucleated and multinucleated giant cells with voluminous eosinophilic ground-glass cytoplasm (finely granular). In early lesions, the predominant infiltrating cells are histiocytes, lymphocytes and eosinophils, with few giant cells, but the giant cell infiltrate quickly follows.

The multinucleate giant cells (the hallmark of the disease) have 3-10 or more nuclei, which may be placed haphazardly, or along the periphery, or clustered in the centre of the giant cell. They contain variable amounts of lipid and free or esterified cholesterol.

Severe involvement of the face may lead to a leonine facies. Does this disease show Köbner (Koebner) response? What are the diseases that may lead to a leonine facies?

The answers are provided below as comments.

This page was last updated in January 2012.

Quiz

2011-10-23T00:23:00.004+02:00

This young adult presented with these scaly lesions (A). On Wood’s light examination (B), these lesions showed yellow fluorescence, and unsuspected similar lesions more widely scattered were revealed as well. What is the fluorescent substance? Is it water-soluble?

Short and long answers are provided below as comments.

Cryotherapy of a cherry angioma

2011-10-20T10:25:00.004+02:00

Cherry angiomas (Campbell de Morgan spots) occur predominantly on the trunk and proximal parts of the limbs.

The freeze time BEGINS at the moment the target area of skin is COMPLETELY frozen (the ice ball). Liquid nitrogen is then sprayed intermittently on the target area to maintain the ice ball at the required margin. Liquid nitrogen spray is halted when the freeze time is up.

In cherry angiomas: Freeze time --> 10 seconds, lesions in general often need more than one freeze. Margin of normal skin also frozen (mm): 1

In larger lesions, the liquid nitrogen spray is directed from the side of the lesion. This allows the extraneous liquid nitrogen to ricochet from the surface of the lesion into the air rather than over normal surrounding skin. If the larger lesion is sprayed directly from the top the extraneous liquid nitrogen will travel across the normal surrounding skin and invariably the physician will terminate cryosurgery before the central base of the lesion has been sufficiently frozen.

The extent of injury is determined by the rate of freezing, the coldest temperature reached, the freeze time and the rate of thawing. Pain peaks during thawing. Use words like discomfort, burn, or pain whenever appropriate when describing the reaction so that the patient will know what to expect.

Immune restoration Klebsiella lymphadenitis

2011-09-02T17:00:00.003+02:00

Gohar A. Immune restoration Klebsiella lymphadenitis. International J STD AIDS 2011; 22:536-7.

Immune restoration syndrome unmasking Klebsiella lymphadenitis was reported in an HIV infected patient for the first time.

Erythema dyschromicum perstans treatment options

2011-08-28T08:05:00.001+02:00

Erythema dyschromicum perstans (EDP) has similarities to lichen planus pigmentosus although the precise relationship of the two conditions has yet to be established.

How to treat it?

There is no established therapy for this condition. Having said so, there are 5 options to be considered.

1) No therapy. The condition is persistent, but may resolve over time. In a series of 4 patients followed up for 2 years in Finland, 3 showed spontaneous resolution.

2) Vitamin A. Lichen planus pigmentosus has responded (50 – 100% clearance in some patients) to vitamin A prescribed in pulses of 100,000 units daily for 15 days (pulses were repeated up to 10 times) in a study. This recommendation is considered by those who reckon that EDP is the same entity as lichen planus pigmentosus.

3) Dapsone 100 mg/day for 8 to 12 weeks might be effective (anecdotal report).

4) Oral steroids might work (anecdotal report).

5) Clofazimine* 100 mg/day for 12 weeks. It seems that clofazimine reduces the inflammatory response in EDP. 4 out of 6 patients showed marked improvement.

*Barabda L, Torres-Alvarez B, Cortes-Franco R et al. Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatosis): the effect of clofazimine therapy. Arch Dermatol 1997; 133: 325–9.

Situations to remember (1)

2011-07-01T08:13:00.000+02:00

I recall seeing a young man who presented with secondary transsexualism of relatively short duration. Later, frank schizophrenia developed and it seemed that the transsexual manifestations were simply part of that illness because they remitted with the treatment of schizophrenia.

Egyptian Medical Practice, reaching the required standards and maintaining them

2011-05-28T19:15:00.008+02:00

Medical Renaissance Initiative
First National Conference on Medical Education Development and Improvement in Standard of Living for Doctors
National Training Institute (NTI), Cairo, Egypt

My Lecture:
Egyptian Medical Practice, reaching the required standards and maintaining them, May 2011

Abstract: The British uniform standards have been compared to the Egyptian variable standards. Whatever the path the Egyptian doctor will choose, certificate of completion of training (CCT) will be awarded by a new national independent professional body when appropriate (in terms of units of study/years/exams) and only then membership can be awarded (e.g. membership of the Egyptian College of Physicians) . To maintain membership continuing professional development (CPD) must be maintained. Different credits of CPD (and how to acquire them), and establishment of a medical protection society have been discussed too.

Relevant links:
1) http://www.dr-academy.com/ar/Recommendations_of_The_First_National_Conference_for_Improvement_of_The_Medical_Education_and_Doctors'_living_Standards.html توصيات المؤتمر القومي الأول لتطوير التعليم الطبي وتحسين أوضاع الأطباء
2)http://www.4shared.com/document/qeGzCMoE/Egyptian_Medical_Practice_reac.html

Perianal haematoma

2011-05-26T12:33:00.003+02:00

This is also called thrombosed external haemorrhoid and it relates anatomically to the veins of the external haemorrhoidal plexus. It is usually consequent upon straining at stool, coughing or lifting a heavy weight (back pressure on an anal venule).

The condition appears suddenly and is very painful. On examination an olive-shaped, blue (resembling a semi-ripe blackcurrant) tense, tender subcutaneous swelling at the anal margin is seen. The haematoma is usually situated in a lateral region of the anal margin. If it is left untreated it may give rise to a skin tag or burst and extrude the clot or bleed. Resolution or fibrosis usually occurs. The condition has been called ‘a 5-day, painful, self-curing lesion’ by Edward Milligan of England.

If the patient presents within the first 48 hours the haematoma may be evacuated under local anaesthesia and the wound is allowed to granulate. If the perianal haematoma is situated anteriorly or posteriorly, it should be treated conservatively because of the liability of the wound in these regions to become an anal fissure.

Pachydermoperiostosis

2011-05-03T20:32:00.004+02:00

Pachydermoperiostosis (Gk pachys thick) is characterized by hypertrophic changes involving primarily the skin (pachydermia) and bones of the extremities (periostosis). 

Primary pachydermoperiostosis (also called primary hypertrophic osteoarthropathy or Touraine–Solente–Golé syndrome) is a rare inherited disorder and occurs predominantly in males. It is sometimes autosomal dominant (with variable expressivity). At other times, it is autosomal recessive. Primary pachydermoperiostosis usually begins soon after puberty and progresses up to 10 years. Exceptionally, it may continue to progress. The skin of the face, forehead and scalp becomes grossly thickened and thrown into folds (coarse facial features, one of the leonine facies). The folding of the scalp produces one of the forms of cutis verticis gyrata. The skin of the hands and feet is also thickened, but often not folded.  Thickening of the phalanges and of the bones of the limbs produces spade-like hands and feet with increased circumference of arms and legs/the fingers and toes are clubbed (related to altered proteoglycan synthesis). Reported associated features include e.g.: acro-osteolysis, sparse facial and pubic hair, gynaecomastia, seborrhoea of the face and scalp, palmoplantar hyperhidrosis, carpal and tarsal tunnel syndrome, and learning difficulties. Arthralgias can be debilitating. There are reports of resolution of the arthralgias when treated with tamoxifen.

Secondary pachydermoperiostosis (also called secondary hypertrophic osteoarthropathy) is usually provoked by serious pulmonary pathology such as bronchial carcinoma and lung abscess. Secondary pachydermoperiostosis occurs predominantly in men aged 30–70 years. The bone changes are the most obvious feature, develop more rapidly, and are usually painful. The skin changes may be absent and are usually relatively mild. When the primary disease is treated effectively, the bone and skin changes will regress. The primary and secondary forms of pachydermoperiostosis must be differentiated by the age of onset, the rate of progression and the presence of underlying pulmonary pathology.

Cranio-osteoarthropathy shares some features with primary pachydermoperiostosis [clubbing and hypertrophic osteoarthropathy], but patients do not have pachydermia. They typically present earlier in childhood and have delayed neurocranial ossification, with delayed closure of the fontanelles, and may also have congenital heart disease. Having said so, the overlap of clinical features suggests that primary pachydermoperiostosis and cranio-osteoarthropathy are allelic in some patients. In acromegaly, the facial skeleton, jaw and skull as a whole are enlarged, and visual defects may be detectable. In thyroid acropachy, enlargement is confined to hands and feet, and exophthalmos and pretibial myxoedema are often present, with other signs of hyperthyroidism.

Primary anetoderma

2011-04-29T18:01:00.012+02:00

Discussed online (synchronous) in April 2011.

The term anetoderma (Gk anetos slack) refers to a circumscribed area of macular atrophy of the skin associated with a loss of dermal substance on palpation and a loss of elastic tissue on histopathological examination.

Primary anetoderma implies that there is no associated, localized, underlying cutaneous disease, whereas true secondary anetoderma can be attributed to some associated condition though the atrophic areas do not always develop at the sites of the known inflammatory lesions.

Primary anetoderma has been divided into the Jadassohn–Pellizari type, in which the lesions are preceded by erythema, and the Schweninger–Buzzi type, in which there is no preceding erythema. The two types of primary anetoderma can coexist in the same patient, and the prognosis and histopathology are identical in both. Thus this differentiation is of historical interest only.

Primary anetoderma is strongly associated with antiphospholipid antibodies with or without a prothrombotic state.

A middle-aged woman presented with skin coloured to pink sac-like protrusions 1–2 cm in diameter, mainly on upper arms and back that have developed over many years and remained unchanged. The lesion surface was thinned and on palpation the examining finger sank into a pit (buttonhole sign). The protrusion reappeared as soon as the pressure from the finger was removed. Coalescence of smaller lesions gave rise to larger protrusions. The lesions arose on clinically normal skin. Few lesions were depressed. There was no history of underlying associated skin disease. Histopathology was consistent with anetoderma. Diagnosis of primary anetoderma was made. The key defect in anetoderma is damage to the dermal elastic fibres (focal elastolysis). Antiphospholipid antibodies have not been detected in the patient. No regularly effective treatment has been found.

British National Formulary (BNF)

2011-03-10T20:12:00.002+02:00

BNF E-Newsletter

BNF Website

BNF for Children (BNFC) E-Newsletter 

BNF for Children (BNFC) Website 

Each new edition of the BNF/BNFC is updated with the latest medicines information and guidance on best medical practice.

Diagnostic Test Performance

2011-01-13T02:25:00.005+02:00

Sensitivity: PID “Positive In Disease” = true positive / all who are really diseased (true positive + false negative by that test)

Specificity: NIH “Negative In Health” = true negative / all who are really disease free (true negative + false positive by that test)

Positive Predictive Value (PPV): true positive / all +ve results by that test in other words true positive + false positive

Negative Predictive Value (NPV): true negative / all –ve results by that test in other words true negative + false negative

In simple words:

Sensitivity: probability of a positive TEST result if the patient is diseased.

Specificity: probability of a negative TEST result if the patient is not diseased.

PPV: probability that a PATIENT tested positive is indeed diseased.

NPV: probability that a PATIENT tested negative is indeed not diseased.

The predictive value of a test not only depends on its sensitivity and specificity, but also on the disease prevalence.

The more prevalent the disease, the higher the positive predictive value of the test and the lower its negative predictive value and vice versa.

This may confuse some, as one might say, why on earth would the prevalence be affecting the whole issue in the population tested?

The following example will clarify it:

Using a test with a sensitivity of 100 % and a specificity of 99 % (i.e. 1 false positive in 100), and screening 1,000 patients, one would expect to see the following:

*In a setting with a high disease prevalence of 10 % (i.e. 100 per 1,000)

100 true positives per 1,000

10 false positives per 1,000 (constant)

Positive predictive value: 100 true positives/110 total positives = 91 %

100 (91 %) of the 110 positive test results are true.

*In a setting with a low disease prevalence of 0.1 % (i.e. 1 per 1,000)

1 true positive per 1,000 (this figure is now significantly lower as the disease is less prevalent)

10 false positives per 1,000 (constant)

Positive predictive value: 1 true positive/11 total positives = 9.1 %

Only 1 (9.1 %) out of 11 positive results is true.
In other words, >90 % of patients tested positive in a low-prevalence population will be actually false positives!

Sexually Transmitted Infections (STI) testing update

2011-01-08T01:47:00.010+02:00

Silver Jubilee ESDV Conference
Marriott Hotel, Zamalek, Cairo, Egypt
Sexually Transmitted Infections (STI) testing update, January 2011.

http://www.4shared.com/document/DvYU6cbH/STI_Testing_Update_2011_2003_v.html

Note that a test of cure is now recommended for ALL cases of gonorrhoea (culture should be delayed for at least 72 hours and NAAT at least 2 weeks after treatment).

This page was last updated in July 2011.

Management of Erectile Dysfunction in hepatic insufficiency

2011-01-08T01:37:00.002+02:00

Egyptian Association for the Study of Gastrointestinal and Liver diseases - Special Interest Group Liver and systemic diseases Conference, Management of Erectile Dysfunction in hepatic insufficiency, October 2009.

http://www.4shared.com/document/n1_E2e8f/Management_of_Erectile_Dysfunc.html

HIV Vaccine and Vaccines in HIV infection

2011-01-08T01:35:00.002+02:00

World AIDS Day (WAD) Conference, Kasr El Aini School of Medicine, Cairo University, HIV Vaccine and Vaccines in HIV infection, December 2007.

http://www.4shared.com/document/gzRgI5Kq/HIV_Vaccine_and_Vaccines_in_HI.html

Abandonment of the name 'laptop'

2011-01-08T01:28:00.008+02:00

Gohar A. Comment on the letter by Bachmeyer, Bensaid and Bégon on Laptop computer as a modern cause of erythema ab igne. J Eur Acad Dermatol Venereol 2009; 23: 1221 - 2.

I recommend the abandonment of the name 'laptop' as a modern word and the use of the plain term 'portable computer' instead for laptop and notebook in view of the health hazards raised and reviewed recently.

Cyclosporin skin darkening

2011-01-08T01:16:00.005+02:00

Gohar A. Comment on the letter by Sadighha and Zahed on hair darkening after treatment with cyclosporin in a patient with psoriasis. J Eur Acad Dermatol Venereol 2009; 23: 862.

A 38-year old female psoriatic patient developed skin darkening on two occasions, one year apart, each after short term course of cyclosporin.

Images (Johns Hopkins University Dermatology Department Image Atlas)

2011-01-08T01:05:00.002+02:00

http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=231093434

Neuropathic hand pruritus

2011-01-08T00:55:00.002+02:00

Gohar A. Neuropathic hand pruritus. Indian J Dermatol Venereol Leprol 2009;75:531-2.

Hand pruritus linked to cervical spine disease was reported for the first time.

Sexually transmitted diseases pattern among men attending a specialised hospital (El-Haud El-Marsoud)

2011-01-08T00:50:00.004+02:00

My thesis dealt with different aspects of sociocultural and socioeconomic factors together with sexual orientation that interacted with sexual attitudes and practices of my patients at the Sexual Health Clinic, El Haud El Marsoud Hospital (a famous tertiary referral centre, with walk-in clinics, for skin and venereal diseases in Egypt established by the British in 1902).

Gohar A. Sexually transmitted diseases pattern among men attending a specialised hospital (El-Haud El-Marsoud). Cairo University, 1993.

Syphilis ranked the second among the diagnosed STDs in this study.

Perforating granuloma annulare

2010-12-10T23:12:00.002+02:00

Different clinical variants of granuloma annulare have been described. The most common form, as its name indicates, is an annular lesion. The annular margin is firm to palpation and may be continuous or consist of discrete or coalescent papules in a complete or partial circle. The epidermis is usually normal, but surface markings may be attenuated over individual papules whose colour may be skin coloured, erythematous, or violaceous. The lesions are usually asymptomatic. Reports of an association with diabetes mellitus are controversial. Several other associations have been reported.

There is overlap between the different variants, and more than one morphologic type may co-exist in the same patient. It must be noted that some lesions enlarge as nodules rather than having an annular morphology. A diagnosis of localized annular granuloma annulare is made on clinical examination. Histopathologic examination is necessary when the presentation is atypical. The clinical diagnosis is definitely difficult when annular lesions are absent.

Perforating granuloma annulare was named by Owens and Freeman in 1971. It exhibits transepidermal elimination of degenerating collagen histopathologically, the so called “necrobiotic collagen” hence classified as a secondary perforating disorder. As the late A Bernard Ackerman used to stress, collagen is a fibre that can undergo degeneration but cannot become necrotic; only cells can undergo necrosis. Perforating granuloma annulare starts clinically as small papules with central umbilications. Lesions may be localized (usually in an acral distribution) or generalized. Perforating granuloma annulare lesions have been present in some of the reported HIV-positive patients with granuloma annulare.

In perforating granuloma annulare there is a superficial area of the so called necrobiosis (better called degenerated collagen, as stated above) and mucin accumulation surrounded by palisading histiocytes, situated beneath a perforation in the epidermis. The so called “necrobiotic material” and mucin are extruded via the perforation. At the margins of the perforation there are varying degrees of epidermal hyperplasia. It should be noted that the epidermal changes are inconspicuous in other variants of granuloma annulare.

Mucinous degeneration of collagen appears bluish or at least shows decrease in the degree of eosinophilic (pink) staining. Mucin can be seen more readily by staining with Alcian blue or colloidal iron.

The differential diagnosis of perforating granuloma annulare includes some dermatoses such as insect bites, molluscum contagiosum, pityriasis lichenoides et varioliformis acuta, transepidermal elimination disorders such as perforating collagenosis and acquired perforating dermatosis, sarcoidosis and papulonecrotic tuberculide. Epithelioid sarcoma may masquerade as perforating granuloma annulare. It should be noted that epithelioid sarcoma that can simulate perforating granuloma annulare, as stated, may also contain mucin. Clues to epithelioid sarcoma include ulceration, cytologic atypia, necrotic foci that include necrotic epithelioid cells and possibly a history of recurrence. Immunohistochemically, epithelioid sarcoma can be distinguished from granuloma annulare by positivity for epithelial membrane antigen and (Cyto)keratin.

The tendency of granuloma annulare to remit spontaneously (but it should be noted that perforating granuloma annulare may leave a hypo- or hyperpigmented scar) complicates accurate assessment of the efficacy of any treatment (such as intralesional triamcinolone 2.5 - 3 mg/mL).

Lymphocytoma cutis

2010-12-06T04:06:00.014+02:00

Lymphocytoma cutis is a benign lymphocyte hyperplasia (B-cell reaction to an antigen such as a red tattoo, Borrelia burgdorferi, injected drug, vaccine) that prefers the head and neck region,and can be associated with sunlight sensitivity. It presents as red or violaceous nodules or plaques and is commoner in females.

It can remain stable or regress over time. Lymphocytoma cutis can be complicated by the development of primary cutaneous B cell lymphoma. Whether the initial diagnosis in such circumstances should have been primary B-cell lymphoma and was missed, or not is uncertain. Lymphocytoma cutis has been classified as a “pseudolymphoma” but it should be noted that the late A Bernard Ackerman used to stress that there is no need to use the term "pseudolymphoma" whenever specific diagnosis can be made.

The lymphocyte infiltrate affects the reticular dermis rather than the subcutaneous fat (In primary B-cell lymphoma the subcutaneous fat may be infiltrated); it spares the papillary dermis forming a grenz zone. It can be wedge shaped, apex of which is directed at the subcutaneous fat but it does not involve it. The infiltrate is considered to be bottom heavy if compared to epidermotropism of mycosis fungoides where there is no grenz zone. Appendages and blood vessels are spared. The infiltrate is polymorphous (lymphocytes, starry sky macrophages (with tangible/stained bodies), eosinophils and plasma cells). The infiltrate shows a mixture of kappa and lambda light chain positive B cells (polyclonal) by immunohistochemical analysis. Normally, B cells undergo gene rearrangement of one of their immunoglobulin heavy chain genes at an early stage in B-cell development. In lymphocytoma cutis, immunoglobulin heavy chain gene rearrangement polymerase chain reaction or southern blot analysis shows polyclonal rearrangement. CD1a+ dendritic cells (originally observed in the infiltrates of cutaneous T-cell lymphoma) are usually abundant by immunohistochemical analysis. It can contain lymphoid follicles (with germinal centres)[in classic cases] resembling the appearance of lymph node. Differentiation from primary B-cell lymphoma (particularly marginal zone lymphoma “MZL”) can be difficult clinically and histopathologically (presence of atypical lymphoid cells would suggest a primary cutaneous MZL).

A spectrum of B-cell neoplasia ranging from polyclonal lymphocytoma cutis (benign) via oligoclonal and monoclonal lymphocytoma cutis into primary cutaneous B-cell lymphoma (malignant) is possible.

Jessner’s benign lymphocytic infiltration (T-cell infiltrate, predominantly in the lower dermis and concentrated tightly around blood vessels and within the lymphocytic infiltrate there is no evidence of follicle formation), tumid discoid lupus erythematosus (basal cell liquefaction degeneration with positive direct immunofluorescence), polymorphic light eruption and insect bites reactions are also among the differential diagnoses.

Treatment modalities (no treatment of proven value unless a cause is found and even then elimination of the cause does not necessarily cures the disease, sunscreens are used if there is sunlight sensitivity) include: intralesional steroid injection, topical steroids, oral hydroxycloroquine, intralesional interferon-alpha-2A, and topical tacrolimus.

Linear porokeratosis

2010-12-04T19:00:00.007+02:00

Linear porokeratosis has been classified as an epidermal naevus but it is still clinically and histopathologically indistinguishable from the lesions of generalized porokeratosis (disorder of keratinization). Linear porokeratosis probably reflects mosaicism for a gene responsible for generalized porokeratosis (autosomal dominant, but CAP syndrome “craniosynostosis – anal anomalies – porokeratosis” appears to segregate as autosomal recessive trait). This mosaicism can be explained by loss of heterozygosity for this dominant gene. Accordingly, an individual with inherited generalized porokeratosis undergoes a somatic mutation affecting the normal allele resulting in clonal loss of heterozygosity. The resultant line has ‘double the dose’ of porokeratosis. Skin cancers in porokeratosis are particularly associated with the linear variant. Overexpression of p53 [tumour suppressor protein (coded for by 17p)] has been reported in linear porokeratosis and in a superimposed squamous cell carcinoma. Many of topical agents, such as imiquimod 5% cream, an immune response modifier that induces cytokines such as interferon-alfa, used in the treatment cause initial inflammation which is followed by resolution.

Linear porokeratosis, with its ‘double the dose’ of porokeratosis, contrasts with the background skin, with its ‘one dose’ porokeratosis. Thus patients with linear porokeratosis can later develop disseminated superficial actinic porokeratosis (DSAP), the commonest presentation of porokeratoses, and children or siblings of patients with DSAP can have linear porokeratosis. Having said so, linear porokeratosis usually occurs sporadically and even many patients with generalised porokeratosis itself have no family history. Linear porokeratosis can exacerbate during episodes of liver failure, and improve following transplantation (unlike generalized porokeratosis that can be provoked by immune suppression).

Almost all epidermal naevi follow the pattern of lines documented by Blaschko from drawings of epidermal naevi. The pattern is attributed to the lines of migration and proliferation of epidermal cells during embryogenesis. The lines do not correspond to any known nervous, vascular or lymphatic structures. The bands of abnormal skin represent clones of cells carrying a mutation in a gene expressed in the skin. Mosaicism impact on the phonotype relates to the proportion of cells that harbour the mutation and their distribution. Mosaic describes an art form in which pictures are produced by joining together tiny pieces of different coloured stone or glass. The term is used in genetics to describe individuals composed of cells of different genotypes, such as patients with Turner syndrome who have both 45, XO and 46, XX cells. The pattern may vary according to cell type and timing of mosaicism. In the skin, genetic mosaicism classically appears as Blaschko's lines.

It should be noted that the term "porokeratosis" was first used by Vittorio Mibelli, Italian dermatologist, in 1893. Porokeratoses are characterised by presence of abnormal (disordered metabolism/increased cell kinetics) clone of keratinocytes showing varying degrees of dysplasia with gradual centrifugal migration of this clone resulting in a keratotic lesion that can progress to overt cutaneous neoplasm. The term “porokeratosis” is a misnomer, as porokeratosis has nothing to do with pores of sweat glands. Actually the lesions may or may not involve the eccrine sweat duct. The characteristic histopathology is seen in the edge of the lesion. The stratum corneum is hyperkeratotic, and at the raised border a column of poorly staining parakeratotic stratum corneum cells, the cornoid lamella, is seen running through the surrounding normal-staining cells. There is hypogranulosis beneath the cornoid lamella. Cornoid lamellae may also been seen in other conditions such as common warts (distinguished by presence of koilocytes), actinic keratosis (distinguished by presence of cytologic atypia) and some ichthyoses.

The ESDV Continuing Professional Development (CPD) Diary Proposal

2010-11-02T06:27:00.020+02:00

The credit requirements for a 5-Year cycle are a total of 250 CPD credits (with the requirement of 50 credits per year). Of the 250 CPD credits, 125 must be external credits (100 clinical external credits and 25 non-clinical external credits). Non-clinical credits concern educational activities that are not directly related to clinical issues.

Internal credits are given for events within the hospital or clinic such as journal clubs, grand rounds, histopathology meetings, local evening medical meetings, audit, managerial work and dealing with ethical and legal issues.

External credits are given for events outside the hospital or clinic such as attending the ESDV conferences and workshops, the ESDV administrative meetings, international conferences, regional clinical/managerial meetings and management courses. 25 external credits (20 clinical external credits and 5 non-clinical external credits) are required per year.

Personal credits relate to study such as private reading of journals and texts, carrying out information searches, writing articles, preparing lectures, CPD activities online, reflective notes writing (clarifying your thoughts after a specific experience), refereeing papers, researching, making presentations at conferences or to examining postgraduates, writing examination questions, dealing with ethical and legal issues and managerial work. Only 10 personal credits may count per year.

Exemption credits may only be granted by the ESDV PER YEAR in exceptional circumstances when a physician is unable to meet the yearly minimum requirements due to illness, maternity leave or long absence from work. In such circumstances, these exemption credits claimed must be made up in subsequent years by completing extra credits as they are not counted towards the 5-Year cycle.

Normally medical practice involves internal, external and personal educational activities with both clinical and non-clinical nature and there should be a balance in the learning methods. In any one-year period, a minimum of 25 credits MUST be external credits "20 clinical external credits + 5 non-clinical external credits"; the minimum remaining credits (25), should be "internal credits + personal credits" or "internal credits + personal credits + external credits". Only 10 personal credits may count toward the total yearly minimum credit requirement as stated above.

Klinefelter’s syndrome

2010-10-27T19:28:00.031+02:00

Klinefelter’s syndrome was named after Harry Klinefelter, an endocrinologist at Massachusetts General Hospital, USA in 1942 who thought it was an endocrine disorder due to lack of a second testicular hormone that he postulated but was unable to identify. The postulated hormone, inhibin, was isolated later.

There are two inhibins (inhibin A and inhibin B), both of which inhibit FSH release by direct action on the pituitary though inhibin B appears to be the FSH regulating inhibin. Inhibin B is a marker of spermatogenesis and Sertoli cell function and is produced by the Sertoli cells and it is the major negative feedback regulator of FSH release. It is undetectable in patients with Klinefelter’s syndrome hence the elevated FSH.  Inhibin B correlates positively with sperm concentration and testicular volume and it correlates negatively with the degree of testicular damage.

The 47, XXY karyotype characteristic of Klinefelter syndrome was first described in Scotland, UK in 1959. XXY karyotype of maternal origin arises by an error at either meiosis I (MI) [commonest source of maternal nondisjunction - ? advanced maternal age] or meiosis II (MII). XXY karyotype of paternal origin arises by an MI error with the formation of XY sperm (? advanced paternal age) because, error at MII forms XX sperm or YY sperm rather than XY sperm.

Klinefelter’s syndrome is the commonest sex chromosome abnormality.

Klinefelter’s syndrome is a classic example of seminiferous tubular sclerosis (where germ cells and Sertoli cells are not identified lining  many sclerotic seminiferous tubules that reveal hyalinising fibrosis with absent spermatogenesis, seminiferous tubules can be completely obliterated).

Germ cell presence and sperm production are variable in men with Klinefelter’s mosaicism. The term mosaic describes an art form in which pictures are produced by joining together tiny pieces of different coloured stone or glass. The term is used in genetics to describe individuals composed of cells of different genotypes, such as  Klinefelter’s mosaicism patients and patients with Turner syndrome who have both 45, XO and 46, XX cells. The pattern may vary according to cell type and timing of mosaicism. Mosaic forms of Klinefelter's syndrome represent mitotic nondisjunction within the developing zygote and are thought to occur in approximately 10% of individuals with this condition. The commonest form observed is 46, XY / 47, XXY mosaicism.

A nonmosaic Klinefelter’s syndrome patient was able to impregnate his wife without assisted reproductive technology then showed severe oligoasthenozoospermia followed by azoospermia*. This and other studies suggest that early (before the age of 35 years) sperm (if detected) retrieval and cryopreservation for future management of infertility in Klinefelter's syndrome should be considered.

The mechanism by which an extra X chromosome renders patients infertile is not known. 

The phenotype is normal (there might be disproportionately long legs, clinodactyly and learning difficulties) before puberty, which starts at the normal age but as a process fails to progress normally.

Adult men with Klinefelter’s syndrome have small firm testicles (testicular volume is rarely over 4 ml). The phenotype can vary from a normally virilised man to one with stigmata of androgen deficiency (Leydig cell function is commonly impaired but Leydig cells are present in normal or increased numbers), including female hair distribution, scanty body hair and long legs (usually tall patients) because of late epiphyseal closure. Patients may develop gynaecomastia (with risk of breast cancer). Ultrasound findings of testicular microlithiasis can be seen in men with Klinefelter’s syndrome.

FSH and LH levels increase to hypergonadotropic levels, inhibin B decreases to undetectable levels, testosterone levels are low or low-normal or low (thus libido can be normal) and oestradiol levels are normal or elevated. The diagnosis is confirmed by chromosome studies.

Reported associated features include e.g. osteoporosis, psychiatric disorders, leg ulcers especially in combination with hyperpigmentation or atrophie blanche (androgens may protect against their development), incontinentia pigmenti (as incontinentia pigmenti trait is usually lethal in males, it has been proposed that the second X chromosome protects against foetal death), and systemic lupus erythematosus [SLE](androgens may normalise SLE haematological and serological abnormalities with clinical remission).

Due to the significant increase of sex chromosomal and autosomal abnormalities in the embryos of Klinefelter patients generated through ICSI, pre-implantation genetic diagnosis (PGD) is strongly advised.

Testosterone replacement is needed as the patient ages and it should be noted that a threshold serum testosterone level is required to achieve full efficacy with PDE5i (phosphodiesterase-5 inhibitors) when required. There is controversy over the indication for testosterone treatment of children with Klinefelter's syndrome (? might improve learning ability).

Yearly follow up of men with Klinefelter’s syndrome is required and androgen replacement therapy should be started when testosterone level is in the range of hypoandrogenism. All men with Klinefelter’s syndrome who undergo testicular biopsy procedures for sperm retrieval need long-term endocrine follow-up.

*Ichioka K, Utsunomiya N, Kohei N, Ueda N, Inoue K, Terai A. Adult onset of declining spermatogenesis in a man with nonmosaic Klinefelter’s syndrome. Fertil Steril 2006; 85(5): 1511.e1-2.

Gonorrhoea

2010-10-26T06:32:00.023+02:00

Gonococcal urethritis (Gonorrhoea). Gonorrhoea owes its name to the Greek physician Galen in the second century AD, as it was then thought that the discharge of urethral pus was seminal fluid-hence being misnamed “gonorrhoea” (Greek, flow of seed). Thus from the very earliest times it was characterised as being “a male disease” and little attention was given to its manifestations in females.

As the concept developed that gonorrhoea resulted from coitus (venery), steps were taken for its control and it is said that in the Middle Ages prostitutes of Paris were quartered in domiciles in the Les Clapiers (les clapiers meaning rabbit hutches, the slang name for brothels in Paris during the Middle Ages) district there, hence the term clap, which is still commonly used to refer to this disease.

Extragenital infections include rectal, pharyngeal and conjunctival infections.

Gonorrhoea has a high infectivity and is easily transmitted before symptoms appear (during the incubation period - the incubation period ranges from 1 to 14 days or even longer in men). In males, the usual presentation is with acute anterior urethritis. There may be slight tender enlargement of the superficial inguinal lymph nodes. Urethritis in men may be divided into gonococcal (gonorrhoea) or non-gonococcal urethritis (NGU); there is considerable overlap and differentiation on clinical grounds alone is not advised. More than 50% of females are asymptomatic (seen as contacts on examination). Symptoms if present (e.g. lower abdominal pain, dysuria, and vaginal discharge) appear within 10 days of infection.

NGU occurring soon after curative therapy for urethral gonorrhoea is called postgonococcal urethritis. NGU can be further classified into Chlamydia trachomatis-positive and Chlamydia trachomatis -negative NGU. There is a proportion of cases of NGU that are not sexually transmitted.

The gonococcus (Neisseria gonorrhoeae) has a predilection for columnar epithelium which is readily available after the organism has gained entrance to the urethra, rectum, pharynx and conjunctiva in both sexes and endocervix and Bartholin’s ducts in females. The infection can spread to other structures lined with susceptible epithelium such as Littré’s glands, Cowper’s glands, prostate gland, seminal vesicles and epididymes in the male and Skene’s glands and Fallopian tubes in the female. The gonococci, with the help of pili and other surface proteins, attach themselves to the epithelial cells. This attachment protects them from being dislodged by the normal flow of urine or mucous secretions. They may also become embedded within the epithelial cells. However, once phagocytosed by neutrophils (microphages), the gonococci may undergo lysis, especially after appropriate antibiotic treatment.

In contrast to female adults, in prepubertal girls the nonoestrogenized alkaline thin immature vaginal mucosa may be infected and colonised with N. gonorrhoeae. A clinician who is examining a prepubertal female for a gonococcal infection should be aware that contrary to female adults, it is not necessary to obtain a specimen from the cervical canal; a specimen from the vaginal canal suffices.

Gonococcal urethritis in boys, vulvo-vaginitis in girls and pharyngeal and rectal infection in both sexes are almost always the result of sexual abuse.

Complications in males include infection of the median raphe, Tysonitis, meatal paraurethral gland abscess, inflammation of Littré’s glands and periurethral cellulitis and abscess, urethral strictures and fistulae, Cowperitis and abscess formation, prostatitis and seminal vesiculitis and epididymitis.

Complications in females include inflammation of the paraurethral (Skene’s) glands, Bartholinitis with abscess formation, and pelvic inflammatory disease (PID). In pregnancy, genital infection is less likely to be complicated by PID (because of thickening of cervical mucus) but other adverse outcomes include e.g. premature rupture of membranes, and postpartum/intrapartum or postabortal endometritis.

Systemic complications include perihepatitis and disseminated gonococcal infection (DGI) (In DGI factors include e.g. serogroup IA-1 & 2/ auxotype AHU [arginine, hypoxanthine, and uracil] dependent). In DGI, the preceding genital infection tends to be asymptomatic and the patient usually presents with mild fever, gonococcal dermatitis especially at extremities, and arthralgia +/- arthritis. AHU/IA-1 & 2 strains accumulate in the population because of their ability to cause asymptomatic infection in men. AHU/IA-1 & 2 strains are particularly susceptible to the penicillins but this varies over time and penicillin resistant strains now play a significant role.

Neonatal gonococcal infection occurs because of exposure in birth canal during labour and includes gonococcal ophthalmia neonatorum, gonococcal arthritis, anogenital infection, pharyngeal infection and other infections such as scalp abscess following trauma to scalp e.g. with intrauterine foetal monitoring.

Five or more polymorphonuclear leucocytes per high power field (in at least five x 1000 oil immersion microscopic fields) in the Gram-stained urethral smear will confirm urethritis and the presence of Gram-negative intracellular diplococci (red kidney bean-shaped organisms) within polymorphonuclear leucocytes (microphages) would enable the physician to make a presumptive diagnosis of gonococcal urethritis. Confirmation is by culture of the organism (also allows sensitivity testing) and differentiation from other Neisseria species by antigenic (monoclonal antibody test) or biochemical testing (gonococcus utilises glucose only). The culture medium is examined at 24 and 48 hours for growth. Growth is optimal at 35–37 °C in a 5% CO2 atmosphere, at a pH of about 6.5–7.5. For urethral specimens from symptomatic men, cultures on selective “antibiotic-containing” (e.g. modified Thayer-Martin medium) and nonselective media are equally sensitive, because the concentration of gonococci in the urethra usually exceeds that of other flora. In contrast, selective media are preferred for culturing the endocervix, rectum, and pharynx, where other, less fastidious bacteria often outnumber N. gonorrhoeae. Test of cure by culture should be delayed for at least 72 after treatment.

Nucleic acid amplification tests (NAATs) are extremely sensitive and will detect very small amounts of DNA or rRNA. Detection does not necessarily indicate that the organism is viable [the test of cure should be delayed for at least 2 weeks after treatment to avoid false positive results]. Using NAATs that exhibit little cross-reactivity reduces false positive results. NAATs are more sensitive than culture and can also be used as diagnostic/screening tests on non-invasively collected specimens (urine in men and self-taken vulvovaginal swabs). NAATs allow dual detection of N. gonorrhoeae and Chlamydia trachomatis too. Urine is a suitable sample in NAAT screening for Chlamydia trachomatis and so may be tested additionally for N. gonorrhoeae in both men and women, but NAATs sensitivity to detect N. gonorrhoeae in women is higher if a self-taken vulvovaginal swab is used. Confirmation of NAAT reactivity by culture is necessary (urine is not considered a suitable sample for culturing of N. gonorrhoeae or Chlamydia trachomatis) and allows antimicrobial sensitivity testing and thus resistant strains can be identified. Resistance may be due to plasmids (circular DNA fragments independent of chromosome) or chromosome mutations.

Gonorrhoea is known to facilitate the transmission of HIV (increase in detectable HIV in urethral/cervical secretions when infection is present). The impact of untreatable gonorrhoea (given the lack of new anti-gonococcal drugs on the horizon, gonorrhoea may eventually become untreatable) on HIV transmission could be enormous in countries with a high HIV prevalence*.

Uncomplicated gonorrhoea responds to a single dose of a suitable antimicrobial (in view of the global increase in multidrug-resistant gonorrhoea, the wisdom of continued single-dose treatment may be challenged and extended treatment may be required), the choice of dose and drug depending on antimicrobial sensitivity. Management of sexually transmitted urethritis includes, screening for other STIs, contact tracing and sexual partner(s) notification, education/counselling, examination, investigation and treatment.

*Lewis DA. The Gonococcus fights back: is this time a knock out? Sex Transm Infect 2010; 86: 415–21.

Lymphomatoid papulosis

2010-10-26T03:43:00.016+02:00

Lymphomatoid (resembling lymphoma) papulosis represents part of a spectrum of primary cutaneous CD30+ lymphoproliferative disorders. Primary cutaneous CD30+ lymphoproliferative disorders consist of a spectrum of conditions; lymphomatoid papulosis and CD30+ large cell lymphomas which are defined on the basis of clinical and pathologic features. Where a distinction cannot be made, patients are designated as ‘borderline cases’.

The CD30 marker was originally identified as a marker on Reed–Sternberg (RS) cells in Hodgkin’s disease. The classical RS cells are multinucleate, nucleoli are large and prominent. If two nuclei are present, one appears to be the mirror image of the other, and this gives an “owl’s-eye” appearance. In the skin, primary CD30+ lymphoproliferative disorders are invariably of T-cell origin (some biopsies may not show a T-cell receptor clonal pattern though). The nodal CD30+ lymphomas can be derived from B, T or null cells.

The term lymphomatoid papulosis was first used in 1968 by Macaulay to describe a ‘self-healing rhythmical paradoxical papular eruption, histopathologically malignant but clinically benign’. Since then, there has been continued discussion as to whether lymphomatoid papulosis is a malignant, a premalignant or a benign condition. Long-term follow-up is necessary in all cases because of the risk of progression to a bona fide lymphoma such as a primary cutaneous CD30+ large cell lymphoma, mycosis fungoides or Hodgkin’s disease in less than 5% of cases. There appear to be no currently available clinical or pathological prognostic markers to indicate whether such progression is likely. There is debate whether these patients actually have lymphoma from the start, which is low grade and may regress, or whether benign disease actually transforms into lymphoma.

Affected patients have recurrent crops of asymptomatic papular or papulonecrotic or nodular lesions predominantly affecting the trunk, although any body site can be involved. The red–brown papules and nodules develop central haemorrhage, necrosis and crusting, and subsequently spontaneously disappear. Healing may leave atrophic (varioliform) scars. Characteristically, skin lesions in different stages of evolution coexist. The disease is chronic and recurrent (waxes and wanes or comes and goes). The cycle recurs every few months, with no obvious initiating factor. The lesions generally occur first in adult life and may recur in crops for up to 40 years or more. Over time, every individual skin lesion will resolve and there may eventually be a persistent remission.

It has been suggested that interactions between CD30 and its ligand (CD30L) may contribute to apoptosis of the neoplastic T cells and the subsequent regression of the skin lesions.

It should be noted that in general cutaneous lymphomas that are CD30+ ab initio are associated with a good prognosis.

Because of the clinical similarities between lymphomatoid papulosis and pityriasis lichenoides et varioliformis acuta, a relationship between the two conditions was initially suggested. Clonal T-cell populations have been demonstrated in skin biopsies of both conditions. Pityriasis lichenoides et varioliformis acuta occurs more often in younger patients, is generally short-lived, does not develop nodular lesions, is more vesicular and less necrotic, and progression to a malignant lymphoma is exceedingly rare, if it occurs at all. CD30 is generally negative and if CD30+ atypical lymphocytes are found they are few and invariably CD8+ while the presence of CD30+ atypical lymphocytes (usually CD4+ but occasionally CD8+) is the hallmark of lymphomatoid papulosis (A and C subgroups, as will be discussed below). It should also be noted that small recurrent lymphomatoid papulosis lesions can be misdiagnosed as folliculitis or arthropod bites for years.

The histopathology of lymphomatoid papulosis in part correlates with the age of the sampled skin lesion.

Lymphomatoid papulosis can be divided on histopathological grounds into A, B and C subgroups.

In the A subgroup, lesions show non-epidermotropic infiltrate with Reed-Sternberg like cells (CD30+), interspersed in an extensive inflammatory infiltrate of lymphocytes, eosinophils, neutrophils, and extravasated red blood corpuscles.

In the B subgroup, lesions show lymphocytic infiltrates showing early infiltration of the basal and parabasal layers of the epidermis. These atypical T lymphocytes are with convoluted (cerebriform) nuclei similar to those seen in mycosis fungoides and are CD30-.

In the C subgroup, lesions show a monotonous population of large CD30+ lymphocytes with an overall pattern suggestive of primary cutaneous CD30+ large cell lymphoma.

Many cases, however, have all types of lesions coexisting simultaneously.

There is no evidence that intensive combination chemotherapy alters beneficially the course of lymphomatoid papulosis, and indeed there are individual case reports suggesting that high-dose intensive chemotherapy may cause transition to a more aggressive CD30+ lymphoproliferative disorder! In the case of cosmetically disturbing lesions (e.g. scarring or many papulonodules), low-dose oral methotrexate (5-20 mg/week) is the most effective therapy for reducing the number of skin lesions.

Favus

2010-10-25T13:11:00.012+02:00

Favus. Trichophyton Schoenleinii infection. Latin: favus, honeycomb (note that kerion, a different form of tinea capitis/barbae, also means honeycomb but in Greek).

Hair invasion results in favus endothrix pattern. The fungus is entirely confined within the hair shaft but does NOT fragment into arthroconidia (thus the infected hair commonly grows to normal lengths). Actually the relatively few hyphae run intact through the hair, forming tunnels within its structure. When first immersed in KOH, air is initially trapped around the hyphae forming the characteristic, long air spaces. These rapidly fill in with KOH, when the hyphae themselves become visible.

The classical picture of tinea capitis caused by this organism is characterized by the presence of yellowish cup-shaped crusts known as scutula. Adjacent crusts enlarge to become confluent and form a mass of yellow crusting (with unpleasant odour). Having said so, there might be less distinctive changes in the early cases (perifollicular redness and some matting of the hair). Seborrhoeic dermatitis with or without alopecia areata, pityriasis amiantacea, psoriasis and impetigo are considered in the differential diagnosis. Scalp demodicidosis mimicking favus has been reported*.

Trichophyton schoenleinii causes a pale green fluorescence of infected hair under Wood’s light. Because fungi growing in culture or on hair in vitro do not fluoresce in this way, the phenomenon must be attributed to some substance (? a pteridine) produced by the interaction of the fungus and the growing hair.

Extensive, patchy hair loss with cicatricial alopecia and atrophy among patches of normal hair may be found in long-standing cases, where much of the hair loss is irreversible. Discoid lupus erythematosus, lichen planus and other causes of cicatricial alopecia are considered in the differential diagnosis.

Favus appears chiefly on the scalp but may affect the glabrous skin and nails. When infected the glabrous skin develops similar yellowish crusts. T. schoenleinii nail invasion (up to 3% of infections) is indistinguishable from other causes of onychomycosis. T. violaceum and M. gypseum infection can produce a favus-like clinical picture. It is interesting to note that favus among the Bantus in South Africa is called witkop (in Afrikaans it means “the white head”). Tinea capitis in general is predominantly an infection of children, although adult cases are occasionally seen. Favus in particular, shows little if any tendency to clear spontaneously at puberty. Tinea capitis may also be seen in adults with AIDS.

Ketoconazole shampoo or selenium sulphide shampoo is helpful to prevent spread in the early phases of therapy, when used in combination with an oral treatment [higher dosage of griseofulvin (20 mg/kg/day) for much longer course may be needed].

*García-Vargas A, Mayorga-Rodríguez JA, Sandoval-Tress C. Scalp demodicidosis mimicking favus in a 6-year-old boy. J Am Acad Dermato. 2007; 57, suppl. 2: S19-S21.

Grafting techniques in the treatment of androgenetic alopecia versus vitiligo

2010-10-22T00:30:00.021+02:00

Androgenetic alopecia

Hair can be redistributed using autografts. This can be performed alone or in combination with reduction of the bald area by excision and closure. The implantation of artificial fibres may be complicated by foreign body reactions and infection and its use has been banned.

On the scalp, and some other regions of the skin, hair follicles are arranged in groups of follicles known as follicular units. Each follicular unit is supplied by a single arrector muscle, which splits to encircle each follicle within the follicular unit.

Follicular Unit Extraction (FUE) is a hair transplant method that involves removing one follicular donor unit (one follicular unit contains some 3 hairs, sometimes emerging from single follicular opening) at a time from the back or sides of the scalp or occasionally from the body (body hair FUE) and placing it into the bald or thinning recipient area of the scalp. A punch with 0.75 mm diameter is used to extract one follicular unit from the donor area. The patient’s natural follicular units in the donor area are first identified; these are then removed from the surrounding skin and then placed in the recipient area taking into consideration some factors like the appropriate density and orientation. These ‘minigrafts’, are manipulated to produce a natural-looking frontal hairline. Maintaining hair follicle unit groupings enhances graft survival too. FUE leaves less noticeable postoperative scarring in the donor area.

There are manual FUE and powered FUE (P-FUE). In P-FUE (preferred method), the speed of the punch blade rotation is usually 700 to 1,500 rpm. The punch blade penetrates the epidermis and dermis and rotation should be stopped when the punch blade reaches the subcutaneous tissue. At this point, the operator senses a loss of resistance. Over-penetration causes hair transection. The graft is then implanted using a pneumatic implanter or forceps. The P-FUE method can be considered to be superior to the conventional manual FUE method in terms of speed and transection rate. With the P-FUE procedure, a 0.8 - 1.0-mm punch blade is generally used, but harvesting is occasionally impossible unless performed with a 1.25-mm punch blade.

FUE can be used alone or can be combined with removing a strip containing a large number of follicular units from the donor area (follicular units are then dissected under microscopes equipped with fibre optics to avoid the application of heat to the follicles).

If FUE is performed long before the ultimate pattern of hair loss is clear and without adjunctive medical therapy to prevent progression of the balding process, an unnatural appearance can evolve over time that may require further surgery to correct.

Vitiligo

Surgical treatments are reserved for cosmetically sensitive sites (results are less good in the extremities and around orifices) in patients in whom there have been no new lesions, no Koebner phenomenon and no extension of the lesion in the previous 12 months.

Autologous epidermal cell suspension containing basal keratinocytes and melanocytes [the dermis of a split-thickness skin graft is separated from the epidermis, and the dermal side of the epidermal layer is manipulated using a blunt forceps to release cells from the basal layer of the epidermis into a solution of normal saline and a suspension is prepared following centrifugation] applied to laser-abraded lesions (transplanting functional melanocytes to the depigmented area after preparation of the affected area with laser peeling of the skin) followed by phototherapy is the optimal transplantation procedure but requires special facilities. Expanding the autologous cells in tissue culture prior to grafting is feasible and can treat larger areas successfully, without the need for additional phototherapy. However, the culturing introduces growth factors leading to uncertain risks and cultures can fail, reducing the value of the procedure.

Split-skin grafting gives much better cosmetic and repigmentation results than minigraft procedures and actually minigraft procedure is NOT recommended due to a high incidence of side-effects (partial take, thick margins and milia) and poor cosmetic results including cobblestone appearance and polka dot appearance (poor colour match). Epidermal blister grafting (transfer of suction blisters) is an alternative transplantation method, which shows evidence of benefit over placebo but gives less good coverage than split-skin grafting or epidermal suspension applied to laser-abraded lesions.

Practically, split-skin grafting is the best option when a surgical treatment is required.

Acne agminata (lupus miliaris disseminatus faciei)

2010-10-20T17:39:00.015+02:00

Acne agminata (lupus miliaris disseminatus faciei). Agminata Latin agmen, clustered, grouped. Acne agminata lesions cluster around the mouth or on the eyelids or on the eyebrows hence the term ‘agminata’. In many cases, the lesions are widely disseminated around the face especially in the “muzzle” area, cheeks, eyelids and eyebrows and forehead hence the term ‘disseminatus’ but in general the lesions tend to appear more on the central face. There are reports of similar eruptions having an extra-facial distribution (spreading to the arms and legs, the trunk or the axillae) but this seems to be very rare.

Lesions present as asymptomatic symmetrical discrete monomorphic smooth-surfaced reddish-brown (somewhat darker in black skin) papules on the central face. The more pustular form of acne agminata is sometimes called “acnitis”. Diascopy (examination of skin with exclusion of blood by firm pressure of glass) of larger lesions often reveals an apple-jelly (yellowish-brown) colour reflecting their granulomatous histopathology, which also shows central caseation. The lesions are not consistently related to hair follicles. Acne agminata is seen mainly in young adults and adolescents of either gender. This eruption tends to be self-limiting, resolving completely over a few months or up to 2 years (or occasionally more). In some cases there is pock-like scarring.

The old view that this condition being a tuberculide was solely histopathological. All other evidence contradicts this view (as there is no relationship to Mycobacterium tuberculosis infection). To avoid linking the condition, that remains of unknown aetiology, to tuberculosis or acne the acronym ‘FIGURE’ (facial idiopathic granulomas with regressive evolution) has been proposed but the full term is cumbersome!

Acne agminata may be mimicked closely by perioral dermatitis that may spread to the eyelids. Moreover acne agminata may be confined, as mentioned above, to the perioral area and can be difficult to distinguish from perioral dermatitis. Histopathologically, the acne agminata lesions are more consistently granulomatous.

It should be noted that granulomatous perioral dermatitis in children (Facial Afro-Caribbean Childhood Eruption "FACE") may represent a juvenile form of perioral dermatitis or of acne agminata. In contrast to perioral dermatitis it does not spare the narrow zone bordering the lips and pustules are not seen. The histopathology has been variously described as showing non-specific inflammation with hyperkeratosis or, more often, as granulomatous, with the inflammatory changes often, but not invariably, being perifollicular.

Rosacea and acne vulgaris are distinguished by their distribution and polymorphous clinical picture though some has classified acne agminata itself as a variant of rosacea (variant of granulomatous rosacea) but acne agminata patients lack a history of flushing, do not have persistent erythema or telangiectasia, have involvement of the eyelids, and the lesions sometimes heal with scarring.

Lewandowsky’s rosacea-like eruption (many do not recognise it as a distinct clinical entity and consider it a variant of rosacea) is distinguished by its peripheral distribution and smaller papules.

Sarcoidosis (especially micropapular sarcoidosis) occasionally presents difficulties. Scattered nodules of lupus vulgaris are less symmetrical and usually less florid. The papular form of granuloma annulare can mimic acne agminata.

Clinically similar lesions to acne agminata due to “metastatic” silicone granulomas have been reported with silicone breast implants. Also, clinically similar lesions to acne agminata have been reported following exposure to zirconium.

The response of acne agminata to tetracyclines has been variable as has the response to isotretinoin. Oral steroids (low-dose prednisolone) may be needed to prevent new lesions from appearing. Daposne appears to shorten the expected natural duration of the disease and prevent the eruption of new papules. In one case the use of clofazimine 100 mg three times weekly was followed by complete resolution within 8 weeks*. *Seukeran DC, Stables GI, Cunliffe WJ, Sheehan-Dare RA. The treatment of acne agminata with clofazimine. Br J Dermatol 1999; 141: 596–7.

Del Castillo syndrome (Sertoli-cell-only syndrome)

2010-10-20T03:25:00.009+02:00

Germ cell aplasia does not represent a diagnosis but it is rather a characteristic histopathologic finding first described by Del Castillo et al. in 1947 which is sometimes referred to by his name. In 1979, Levin described germ cell aplasia with focal spermatogenesis where a variable percentage of seminiferous tubules contain germ cells. It is important to discriminate between both (by repeated testicular biopsies if the patient desires so) in view of ICSI (IntraCytoplasmic Sperm Injection) / IMSI (Intracytoplasmic Morphologically selected Sperm Injection).

No germ cells are present in the seminiferous tubules but Sertoli cells are present (hence the name Sertoli-cell-only syndrome / Johnsen Score 2: no germ cells - Sertoli cells only). The Sertoli cell nucleus is typically triangular or ovoid in shape with a prominent nucleolus. Sertoli cells with their apical aspects displaced to one side “wind swept tree tops” are seen. Tunica propria and basement membranes are not appreciably thickened, and the seminiferous tubules are of normal or slightly decreased diameter.

The testes of men with Del Castillo's syndrome could be classified into three groups, according to the Sertoli cell type present: mature, dysgenetic (with immature nucleus) and involuting cells (with irregularly outlined nucleus). It is unknown whether this classification has a correlation with Inhibin B levels. In Del Castillo’s syndrome serum FSH is most often elevated.

Inhibin B is a marker of spermatogenesis and Sertoli cell function and is produced by the Sertoli cells. It is the major negative feedback regulator of FSH release. Inhibin B correlates positively with sperm concentration and testicular volume. Inhibin B correlates negatively with the degree of testicular damage.

Mostly germ cell aplasia is congenital (primary) and is associated with primary infertility. For some reason the germ cells do not migrate into or survive in the seminiferous tubule. Microdeletions of the Y chromosome are an important genetic cause of congenital germ cell aplasia. Germ cell aplasia can be acquired (secondary) through endogenous (e.g. maldescended testes) and exogenous (e.g. radiotherapy) damage of sufficient magnitude to destroy the germ cells without destroying the Sertoli cells.

It should be noted that when bilateral testicular biopsies show germ cell aplasia the patient can have oligozoosermia and not necessarily azoospermia as shown in a retrospective review*, hence the importance of clinicopathologic correlation.

*Bettocchi C, Parkinson MC, Ralph DJ, Pryor JP. Clinical aspects associated with Sertoli-cell-only histology. Br J Urol. 1998; 82(4): 534-7.

Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans)

2010-10-20T01:24:00.009+02:00

Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans), sometimes classified as a disorder of keratinization, is one of the four conditions that are regarded as primary perforating disorders. Having said so, the nature/number of perforating disorders is uncertain. The epidermal involvement may be secondary to dermal disease and hence they are probably not true disorders of keratinization.

Kyrle’s disease lesions present as pruritic keratotic papules/nodules developing on the limbs and trunk. The plug protrudes from a crateriform depression from which it can be removed. In Flegel’s disease (hyperkeratosis lenticularis perstans), an autosomal dominant condition, there are also keratotic papules but they have polygonal margins (cornflake sign) and these differ from Kyrle’s disease lesions.

Many dermatoses occasionally exhibit the phenomenon of transepithelial elimination, in which material from the dermis is extruded through the epidermis to the exterior with little or no disruption of the surrounding structures. The extruded material may include inflammatory cells, red blood corpuscles, microorganisms and extracellular substances, such as mucin or altered connective tissue components. In most of these conditions, the elimination is secondary to some underlying disease (hence called secondary perforating disorders), such as perforating granuloma annulare where material in the centre of a palisaded granuloma seated immediately beneath the epidermis is deported from the skin. Occasionally, a chemical that has been applied to the skin topically or by intradermal injection can be eliminated by the transepidermal route to produce a perforating disorder.

There are four conditions that are regarded as primary perforating disorders: Kyrle’s disease, perforating folliculitis, reactive perforating collagenosis and perforating serpiginous elastosis (also called elastosis perforans serpiginosa). It is possible that these primary perforating disorders might be due to defects in the epidermal keratinocytes, hair follicle, collagen and elastic fibres, respectively, with the elimination being the final pathway but it should be noted that in perforating folliculitis, the follicular epithelium shows one or more perforations INTO the dermis. Moreover, perforating folliculitis might not vary sufficiently from any folliculitis in which the follicles have ruptured hence considered by many as non specific entity.

Perforating serpiginous elastosis is easily distinguished from other perforating disorders when characteristic serpiginous lesions are present (in contrast to individual papules in the latter) and by the finding of elastic fibres in the perforating tissue on histopathology but a similar histopathological appearance can occur in the acquired reactive perforating dermatosis (see below) .

These four conditions appear to be separate entities when they occur outside the setting of renal failure and/or diabetes mellitus. There have been numerous reports of these four perforating dermatoses occurring in the setting of renal failure and/or diabetes mellitus (largely on the basis of the histopathologic findings). The four conditions may overlap in such circumstances (e.g. both collagen and elastic fibres can be extruded in the same patient) and the name acquired reactive perforating dermatosis [perforating substances: Necrotic material – collagen – elastic tissue) has been suggested. It must be noted that Kyrle’s disease was first described in a young diabetic female and some actually consider Kyrle’s disease to be synonymous with acquired perforating dermatosis.

The late A Bernard Ackerman used to stress that the perforating concept is not a requisite for accurate morphologic diagnosis and does not illuminate the mechanisms that underlie those diseases. He actually considered Kyrle’s disease to be akin to suppurative folliculitis that induces intense pruritus. Intense pruritus leads in turn to prurigo nodularis superimposed on a resolving folliculitis.

Some add a fifth disease to the primary perforating disorders, that is perforating periumbilical calcific elastosis with its periumbilical keratotic papules (perforating substance is calcified elastic tissue), a grinding sound can be heard on performing a biopsy!

In most cases of acquired perforating dermatosis, lesions could be cleared by treatment with potent topical or intralesional steroids, some patients improve spontaneously though. Topical tretinoin may reduce the lesions.

Acne fulminans (acute febrile ulcerative acne)

2010-10-19T03:58:00.009+02:00

Acne fulminans (acute febrile ulcerative acne) seems to be immunologically induced systemic disease, in which the offending antigen is P.acnes [immediate and delayed (types III and IV) hypersensitivity reactions].

The patients are predominantly young males who quite suddenly develop extensive inflammatory lesions (oozing friable plaques with haemorrhagic crusts) especially on the trunk. The often ulcerated lesions can lead to significant scarring. The affected patients often have typical mild to moderate acne prior to the onset of acne fulminans.

Many associated features have been reported e.g. fever, polyarthropathy, bone pain (aseptic osteolysis), painful splenomegaly, erythema nodosum, marked leukocytosis, anorexia and general malaise. Blood cultures are universally sterile. The prognosis for bone lesions is good, and chronic sequelae, if any, are mild, leading to mild sclerosis and hyperostosis.

Testosterone treatment, anabolic steroids (some athletes abuse them), and oral isotretinoin may induce acne fulminans. One case has been reported in a young man with late-onset congenital adrenal hyperplasia. An acne fulminans-like picture has been reported in association with Epstein–Barr virus infection. Thus, patients with a sudden exacerbation of previously relatively mild acne should be checked for infectious mononucleosis.

Oral prednisolone therapy is required urgently with other medications such as salicylates/ non-steroidal anti-inflammatory drugs and oral antibiotics. Oral low dose isotretinoin is cautiously introduced after about 4 weeks of oral prednisolone and gradually increased as tolerated, according to clinical response and the daily dose of prednisolone is slowly decreased as tolerated.

Topically crusts are removed by soaking the skin with emollient oil followed by the use of a potent steroid/antiseptic cream for 2 to 3 weeks; occasionally with intralesional steroid.

It should be noted that oral isotretinoin itself in general can initially induce inflammatory flares of acne, occasionally leading to acne fulminans as stated above and mild flares do not require modification of the oral dose and improve spontaneously but severe episodes should be treated with systemic corticosteroids, and dose reduction or discontinuation of isotretinoin may be required.

Isotretinoin in combination with dapsone has been used successfully to treat acne fulminans associated with erythema nodosum.

Few patients develop severe vasculitic pyoderma gangrenosum-like lesions (vasculitic acne).

SAPHO syndrome is manifested by synovitis, acne, pustulosis, hyperostosis, and osteitis. It is predominantly associated with hyperostosis of the anterior chest, palmoplantar pustulosis, hidradenitis suppurativa, and acne fulminans.

An autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum and severe acne (PAPA syndrome) was described in 1997.

Noma (cancrum oris)

2010-10-19T03:41:00.003+02:00

Noma (Gk nomē feeding; related to Gk nemein to devour). The Greek origin of the word reflects the rapid progression of this condition. It is also called cancrum oris (cancrum: L crab, eroding ulcer).

Essentially it is a gangrenous stomatitis, which starts in the mouth as a gingival lesion and rapidly destroys both the soft and hard tissues of the mouth and face. It predominantly affects malnourished or immunocompromised young children and is mostly encountered in developing countries. Nigeria probably has the highest incidence. Failure to adequately treat acute necrotizing gingivitis may lead to noma in malnourished or immunocompromised patients. In the developed world, noma is typically seen in immunocompromised persons such as those with HIV infection and leukaemia.

Noma neonatorum is thought to be a related but separate entity from noma. Noma neonatorum is a gangrenous disorder of the nose, lips, mouth, perianal area and, occasionally, the scrotum and eyelids, occurring in low birth weight and/or premature malnourished neonates almost exclusively in the developing countries and it is usually related to Pseudomonas aeruginosa septicaemia. It is almost invariably lethal in the absence of appropriate antibiotic treatment. A form of noma neonatorum has been seen in Native American infants who present with perineal and oral ulcerations in the setting of severe combined immunodeficiency (immunodeficiency of both B and T lymphocytes). Because most cases of noma neonatorum are caused by Pseudomonas aeruginosa, it is possible that this represents a severe form of ecthyma gangrenosum.

Noma appears to be a polymicrobial infection with Prevotella intermedia and Fusobacterium necrophorum being the two most frequently isolated organisms. Although organisms are isolated from the lesions, there is a low likelihood of transmissibility (groupings of noma seem to be more associated with common risk factors rather than true transmission).

The presenting feature is red or purplish-red spot usually on the gingiva, which enlarges and ulcerates rapidly, spreading to the labiogingival or mucobuccal fold, and exposing the underlying bone. There is pain (usually with foetor and cervical lymphadenopathy). A blue–black area of discolouration appears on the skin overlying the intraoral lesion and leads to a perforating wound. As it becomes black, this necrotic zone expands and forms a classic cone shape, cone gangreneux, with internal destruction greater than external involvement. Sequestration of the exposed bone and loss of teeth are rapid and then the wound heals slowly by secondary intention, often leaving a defect. In former times, noma was often a lethal condition.

Gangrenous stomatitis does not respond readily to treatment unless the underlying disease is controlled. The wound should be cleaned regularly with chlorhexidine and/or saline and/or hydrogen peroxide. A soft cotton gauze or tulle gras dressing may be used but changed frequently. Any loose slough, loose teeth and bony fragments should be removed (but invasive intraoral procedures are contraindicated). Parenteral fluids should be given to correct any dehydration and electrolyte imbalance. Some recommend broad-spectrum antibiotics, whereas others believe that metronidazole is adequate as anaerobic organisms predominate.

Healing lesions of noma are difficult to manage because of extensive fibrous scars. These scars can lead to strictures of the mouth, severe dental malposition, defective speech, and even complete closure of the mouth from contractures.