Sunday, 17 October 2010

Erythema elevatum diutinum

Erythema elevatum diutinum (a small vessel vasculitis, also a neutrophilic dermatosis). Latin diutinum, of long duration.

Lesions most commonly appear chronically in a symmetrical fashion over the dorsa of the hands, the knees, buttocks and Achilles tendons in the form of red-violaceous to red-brown plaques.

The plaques show histopathological evidence of leukocytoclastic vasculitis. In addition to the characteristic polymorphonuclear fragmentation, endothelial swelling, extravasation of erythrocytes and fibrinoid necrosis of vessel walls, dermal nodules of the disease contain spindle cells and fibrosis.

The disease is now recognized as one of the defined reactive dermatoses associated with HIV. Lesions with a prominent vascular component, especially in an HIV-infected patient, might be mistaken for Kaposi's sarcoma or bacillary angiomatosis. Erythema elevatum diutinum has also been associated with other diseases such as multiple myeloma, syphilis and rheumatoid arthritis.

Dapsone has been considered the treatment of choice but patients frequently relapse upon discontinuation of the medication. 

1 comment:

  1. Note that:

    1) The clinical differential diagnosis of erythema elevatum diutinum depends upon the stage of the lesion [ earlier lesions e.g. Sweet's syndrome - older lesions e.g. granuloma annulare].

    Apart from the histopathology, one should consider the character, distribution and persistence of erythema elevatum diutinum.


    A) Erythema elevatum diutinum is differentiated from Sweet’s syndrome by the character of the lesions and their distribution. Clinically the lesions in Sweet’s syndrome are acute, more often asymmetrical and located on the arms, face and neck. In contrast, erythema elevatum diutinum lesions are chronic, symmetrical and classically located over the dorsum of the hands and knees, buttocks, and Achilles tendon. Although leukocytoclastic vasculitis has now been reported as a possible feature of Sweet’s lesions, it is not always present and the fibrosis seen in lesions of erythema elevatum diutinum correlates with the clinical chronicity.

    B) Erythema elevatum diutinum is differentiated from granuloma annulare by the deeper colour and persistence of the lesions.

    2) Histopathologically erythema elevatum diutinum can be difficult to distinguish from cutaneous small vessel vasculitis but the clinical picture differs from that of other chronic small vessel vasculitis syndromes. As Professor Rona Mackie (Glasgow, Scotland) used to stress, “do not be misled by the artificial description and differential diagnosis in dermatopathology textbooks”, “the rule is sort out problems first clinically”.

    3) Raised IgA ANCA levels in some cases of erythema elevatum diutinum might be of some diagnostic value.


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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