Genital ulcers: do not forget BehÃ§et disease
Susana Maia, Alexandra Martins, Celeste Santos, FranÃ§ois Alves
Correspondence to Dr Francois Alves, email@example.com
A 22-year-old, previously healthy, woman was referred to our department for genital ulcers present for more than 1 year, with no resolution to the various treatments prescribed. At physical examination, the patient presented a painful ulcer with a central necrotic base and clean margin about 10 mm in diameter on the right labia majora (figure 1) and a similar lesion at the cervix (figure 2). Multiple painful aphthous like ulcers morphologically similar to genital ulcers of the oral cavity (figures 3 and 4) and folliculitis-like lesions on the legs (figure 5) were found. The diagnostic exams revealed only an elevated erythrocyte sedimentation rate. As this patient met the criteria of the International Study Group for [BehÃ§et] disease, based on the occurrence of symptoms and signs that are compatible with the disease, the diagnostic was made. The criteria include recurrent oral ulceration with two of the following four criteria: recurrent genital ulcerations, eye lesions (uveitis or retinal vasculitis), skin lesions (erythema nodosum or foliculitis) and or positive pathergy test (Occurrence of a small red bump or sterile pustule to any intracutaneous insult). The investigation shows no involvement of cardiovascular, renal, gastrointestinal, pulmonary, urologic, joint or central nervous systems. Treatment was established by the Autoimmune Disease Unit and resolution of symptoms was achieved with the administration of corticotherapy and [colchicine]. Two years later, the patient had an uneventful pregnancy resulting in a normal infant.
Figure 5 Folliculitis like lesions on the legs.
- Most genital ulcers found in the young women are caused by genital herpes, syphilis, lymphogranuloma venereum, granuloma venereum or chancroid. Nevertheless, this may be the first manifestation of a systemic disorder.
- BehÃ§et s disease is most common along the Old Silk Route , which spans from Japan and China in the Far East to the Mediterranean region and was named after the Turkish dermatologist Hulusi BehÃ§et, who first described the triad of recurrent oral aphthous ulcers, genital ulcers and uveitis. The aetiology is not known but immunogenetics, immune regulation, vascular abnormalities or bacterial and viral infection may have a role in BehÃ§et s disease development by triggering a profound inflammatory response in a genetically susceptible host. Manifestations of BehÃ§et s disease may occur at many sites throughout the body including frequently the skin, eye, heart, lung, kidney, gastrointestinal tract, joint, blood vessels and central nervous systems. The treatment remains predominantly empirical and the primary goals of management are symptom control, early suppression of inflammation and prevention of end-organ damage. The treatment options are anti-inflammatory agents and immunosuppressant s drugs, used in combination in order to maximise efficacy while minimising side effects. The spectrum of clinical manifestations requires close multi-disciplinary intervention for optimal care.
- This case highlights the importance of recognition of BehÃ§et s disease as a cause of genital ulcer in order to quickly establish the diagnosis and institute appropriate therapy.
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