BERLIN – Many of the classic manifestations of Behçet’s disease show a significant and previously unappreciated gender-based difference in expression, judging from the results of a new study of more than 10,000 patients.
For example, the most common form of skin involvement – erythema nodosum – is more common in women than men with Behçet’s disease, whereas papulopustular lesions and pseudofolliculitis occur more often in men with this highly variable multisystem disorder, Dr. Alfred Mahr reported at the annual European Congress of Rheumatology.
Dr. Alfred Mahr
Dr. Mahr, chairman of the internal medicine department at Saint-Louis Hospital in Paris, presented a meta-analysis of the published literature on Behçet’s disease supplemented by new and unpublished data on 721 patients in the German Registry for Adamantiades-Behçet’s Disease for 1990-2011. All told, the analysis included 30 published studies, which, along with the unpublished data, comprised more than 10,000 affected patients.
The most common expression of Behçet’s disease was genital ulcers, affecting 79% of subjects, closely followed by skin involvement, at 77%. Erythema nodosum was present in 53% of patients, papulopustular skin lesions in 38%, and pseudofolliculitis in 29%.
Other frequent manifestations of Behçet’s disease included eye disease, affecting 49% of subjects, joint disease in 40%, major blood vessel involvement in 9.5%, and either deep vein or superficial thrombophlebitis in 31%.
Joint involvement was present in half of men with Behçet’s disease, compared with more than 60% of women. Even more striking was the gender difference in major vessel involvement, which affected roughly 30% of men and 10% of women. And cardiac involvement was documented in 14 men but just 1 woman.
These observations on gender differences in Behçet’s disease are important for two reasons, Dr. Mahr said: They may help in the differential diagnosis of this multisystem disorder, and they could shed light on disease pathogenesis. For example, Turkish investigators previously identified testosterone as having potentially relevant effects on neutrophil production (Clin. Exp. Rheumatol. 2007;25(4Suppl 45):46-51).
Dr. Mahr reported having no financial conflicts.