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Skin-nontumor / Clinical Dermatology

Other dermatoses

Behcet’s disease

Reviewer: Nat Pernick, M.D., PathologyOutlines.com, Inc. (see Reviewers page)
Revised: 19 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Clinical features
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● Chronic condition with canker sores or ulcers in mouth and genitals and inflammation in parts of eye (National Institutes of Health)
● May also cause arthritis, skin problems, inflammation of digestive tract and CNS
● Most common along “Old Silk Route” from Japan and China to Mediterranean; rare in US (Johns Hopkins)
● Some skin lesions resemble acne but occur throughout body; also erythema nodosum with ulceration
● Aphthous ulcers in mouth (lips, tongue, inside cheek) of almost all patients; are numerous, frequent, large and painful
● May be fatal due to ruptured aneurysms or severe neurological complications

Diagnosis
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● Oral aphthus ulcers and at least 2 of the following: (1) genital aphthae, (2) synovitis, (3) posterior uveitis, (4) cutaneous pustular vasculitis, (5) meningoencephalitis, (6) recurrent genital ulcers, and (7) uveitis in the absence of inflammatory bowel disease or collagen-vascular disease (eMedicine, Lancet 1990;335:1078)

Case reports
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● 34 year old man with aphthous stomatitis, then painful erythematous nodules on lower extremities (Dermatol Online J 2010;16:18)

Clinical images
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Erythematous papules and pustules resembling acne

Calf and abdomen

Erythematous plantar maculae

Micro description
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● Leukocytoclastic vasculitis and panniculitis
● Also superficial and deep perivascular infiltrates of lymphocytes and neutrophils
● Some vessels contain thrombi
● Also suppurative folliculitis, intraepidermal or subepidermal vesicles

Micro images
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Disrupted folliculosebaceous unit surrounded by neutrophils, histiocytes and foci of fibrosing granulation tissue; neutrophils permeate the infundibulum and overlying mounds of parakeratosis; leukocytoclasis is present, but no vasculitis or thrombosis

Endothelial cell swelling and perivascular inflammatory cell infiltrate

Differential diagnosis
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● Localized chronic fibrosing vasculitis: different clinical findings

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Behcet’s disease

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