A Case of HLA-B51 Positive Mucocutaneous Variant of Behçet’s Disease in a Young Indian Male: A Case Report

Abstract

Background: Behçet’s disease is a rare, systemic inflammatory disorder of unknown etiology affecting the mucocutaneous, vascular, skeletal, ophthalmic, gastrointestinal, and neurological systems. While the exact etiopathogenesis of Behçet’s disease is yet to be established, numerous studies have supported a strong possibility of underlying genetic factors. It is comparatively more common in Turkey, the Middle East, and Mediterranean regions, and only a few cases have been reported from the Indian subcontinent so far. Although several immunological and genetic associations have been suggested, the diagnosis of Behçet’s disease remains primarily clinical and of exclusion.

Case Report: In this report, we describe the case of a 22-year-old Indian male who developed multiple aphthous ulcers over his tonsillar pillars and pharyngeal wall following an episode of acute tonsillopharyngitis. Over the course of the next few days, he reported the presence of a genital ulcer and papulopustular lesions over his chest, back and face, eventually prompting a diagnosis of Behçet’s disease. He was treated with oral colchicine and was found to be in remission during a six-week follow-up.

Conclusion: This case emphasizes the importance of awareness regarding Behçet’s disease among clinicians in India and warrants further studies on the epidemiology, immunopathogenesis, and management protocols of Behçet’s disease, especially in this country for a better understanding of its prevalence, manifestations, and disease course.