Fever, arthritis, and painful skin nodules

Answer Behçet’s disease, a type of vasculitis that most commonly affects people aged 20-40.2 The International Study Group diagnostic criteria3 include recurrent oral ulceration (more than three episodes in a year) plus two of four other characteristic findings: genital ulceration; eye lesions (uvei...

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Bibliographic Details
Published in:BMJ (Online) 2021-09, Vol.374, p.n1956-n1956
Main Authors: Lin, Yu-Pei, Chiou, Chi-Sheng
Format: Article
Language:English
Subjects:
Arthritis
Behcet's syndrome
Biopsy
Endgames
Erythema
Erythema induratum
Erythema nodosum
Fever
Herpes simplex
Hip
Histocompatibility antigen HLA
Infections
Inflammation
Joint diseases
Nodules
Patients
Retina
Skin diseases
Skin lesions
Steroids
Streptococcus infections
Uveitis
Vasculitis
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Summary:Answer Behçet’s disease, a type of vasculitis that most commonly affects people aged 20-40.2 The International Study Group diagnostic criteria3 include recurrent oral ulceration (more than three episodes in a year) plus two of four other characteristic findings: genital ulceration; eye lesions (uveitis/retinal vasculitis); skin lesions (erythema nodosum/pseudofolliculitis/papulopustules/acneiform nodules); or a positive pathergy test. Arthritis affects the medium and large joints (eg, wrists, hips, knees, and/or ankles) in approximately half of patients with Behçet’s disease.1 It is most prevalent in Turkey (80 to 370 cases per 100 000) with prevalence ranging from 13.5 to 35 cases per 100 000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia.2 In the US and Europe it is from 0.12 to 7.5 cases per 100 000.4 The cause of Behçet’s disease is still unknown, but herpes simplex virus, streptococcus, and gene sensitivity of HLA-B51 serotype are believed to be contributing factors.5 Owing to its varied clinical presentations, diagnosis and treatment of Behçet’s disease are sometimes delayed. Differentials of erythema nodosum include subcutaneous infections, erythema induratum, polyarteritis nodosa, subcutaneous cancers, pancreatic panniculitis, and α-1 antitrypsin deficiency.6 Learning points Raised temperature, ESR, and CRP could be caused by inflammation if infection is ruled out Consider erythema nodosum when acute tender erythematous nodules develop (especially on the legs) Search for underlying disease when erythema nodosum is present Consider Behçet’s disease in patients with recurrent oral ulcerations who have genital ulcerations, uveitis, retinal vasculitis, erythema nodosum, pseudofolliculitis, papulopustules, acneiform nodules, arthritis, and/or a positive pathergy test.
ISSN:1756-1833
1756-1833
DOI:10.1136/bmj.n1956