Wednesday, 20 October 2010

Acne agminata (lupus miliaris disseminatus faciei)

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 (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!

Note that perioral dermatitis spares the narrow zone bordering the lips

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. Oral daposne appears to shorten the expected natural duration of the disease and prevent the eruption of new papules. Topical dapsone has also been used successfully and might be a viable treatment option for acne agminata which is often difficult to treat. 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. 

This page was last updated in September 2018.

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Main Works of Reference List (The first eight are my top favourites)

  • British National Formulary
  • British National Formulary for Children
  • Guidelines (BAD - BASHH - BHIVA - Uroweb)
  • Oxford Handbook of Genitourinary Medicine, HIV, and Sexual Health
  • Oxford Handbook of Medical Dermatology
  • Rook's Textbook of Dermatology
  • Simple Skin Surgery
  • Weedon's Skin Pathology
  • A Concise Atlas of Dermatopathology (P Mckee)
  • Andrews' Diseases of the Skin
  • Andrology (Nieschlag E FRCP, Behre M and Nieschlag S)
  • Bailey and Love's Short Practice of Surgery
  • Davidson's Essentials of Medicine
  • Davidson's Principles and Practice of Medicine
  • Fitzpatrick's Colour Atlas and Synopsis of Clinical Dermatology (Klaus Wolff FRCP and Richard Allen Johnson)
  • Fitzpatrick’s Dermatology in General Medicine
  • Ganong's Review of Medical Physiology
  • Gray's Anatomy
  • Hamilton Bailey's Demonstrations of Physical Signs in Clinical Surgery
  • Hutchison's Clinical Methods
  • Lever's Histopathology of the Skin
  • Lever's Histopathology of the Skin (Atlas and Synopsis)
  • Macleod's Clinical Examination
  • Martindale: The Complete Drug Reference
  • Oxford Handbook of Clinical Examination and Practical Skills
  • Oxford Textbook of Medicine
  • Practical Dermatopathology (R Rapini)
  • Sexually Transmitted Diseases (Holmes K et al)
  • Statistics in Clinical Practice (D Coggon FRCP)
  • Stockley's Drug Interactions
  • Treatment of Skin Disease: Comprehensive Therapeutic Strategies
  • Yen & Jaffe's Reproductive Endocrinology