Skin nontumor
Dermal non-granulomatous granulocyte-rich reaction patterns
Behçet disease
Author: Nat Pernick, M.D.
Topic Completed: 1 July 2011
Minor changes: 10 November 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Behçet disease [title] skin
Table of Contents
Clinical features | Diagnosis | Case reports | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Pernick N. Behçet disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorbehcetdisease.html. Accessed January 20th, 2021.
Clinical features
- Chronic condition with canker sores or ulcers in mouth and genitals and inflammation in parts of eye (National Institutes of Health: Behçet disease [Accessed 24 August 2018])
- 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 U.S.
- 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
- Oral aphthus ulcers and at least two of the following (eMedicine: Dermatologic Aspects of Behcet Disease [Accessed 24 August 2018], Lancet 1990;335:1078):
- Genital aphthae
- Synovitis
- Posterior uveitis
- Cutaneous pustular vasculitis
- Meningoencephalitis
- Recurrent genital ulcers
- Uveitis in the absence of inflammatory bowel disease or collagen-vascular disease
Case reports
- 34 year old man with aphthous stomatitis, then painful erythematous nodules on lower extremities (Dermatol Online J 2010;16:18)
Clinical images
Images hosted on other servers:
Erythematous papules and pustules resembling acne
Erythematous plantar maculae
Microscopic (histologic) description
- 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
Microscopic (histologic) images
Images hosted on other servers:
Disrupted folliculosebaceous unit surrounded by neutrophils
Endothelial cell swelling
and perivascular
inflammatory
cell infiltrate
Differential diagnosis
- Localized chronic fibrosing vasculitis: different clinical findings
