Pathology of Skin Manifestations in Behçet Disease
摘要
Behçet disease (BD) has no strict pathognomonic features on histopathology, while it has a rather wide spectrum of microscopic changes. Mucocutaneous lesions are the hallmark of the disease, including oral and genital/extragenital ulcers, papulopustular lesions, erythema nodosum (EN)-like lesions, superficial thromboflebitis, pathergy test mainly. Especially in early lesions, neutrophil-predominating infiltrate is common, while in later stages, lymphohistiocytic infiltration is seen. Various vascular changes, such as leukocytoclastic vasculitis, lymphocytic vasculitis, Sweet-like vasculitis, vessel wall thickening, and occlusive vascular reactions accompany the inflammatory cell infiltration. Papulopustular lesions are either follicular or non-follicular. Panniculitis due to BD usually reveals neutrophilic lobular panniculitis, mostly together with vascular changes. Other reported associations of BD are pyoderma gangrenosum-like, erythema multiforme-like, pernio-like lesions, neutrophilic eccrine hidradenitis, bullous lesions due to necrotizing vasculitis, and palpable purpura.