Verma Ghanshyam K, Tegta Gita R, Sharma Anuj, Kaur Mohitpreet, Sharma Sudarshan
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.
Indian Dermatol Online J. 2019 Sep 26;11(1):87-90. doi: 10.4103/idoj.IDOJ_83_19. eCollection 2020 Jan-Feb.
Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. It has two subtypes: Hallopeau variant and Neumann variant. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue, is a well-known sign in pemphigus vegetans. We are presenting an unusual case of pemphigus vegetans with extensive involvement of mucosae, trunk, extremities including both extensors and flexural areas with verrucous paronychia and fissured tongue. During clinical exam we recognized multiple cutaneous vesicles, erosions and ulcers on mucosal sites. Clinical, histopathological, and direct and indirect immunofluorescence findings were compatible with pemphigus vegetans. Patient had completed her family so treated with intravenous dexamethasone-cyclophosphamide pulse (DCP) regimen with excellent response.
增殖性天疱疮是寻常型天疱疮的一种罕见变异型，其特征为脓疱和/或乳头瘤样赘生物，好发于皮肤褶皱部位和口周区域。它有两个亚型：Hallopeau型和Neumann型。脑回状舌是增殖性天疱疮的一个著名体征，表现为舌背有典型的脑回样沟回形态。我们报告一例罕见的增殖性天疱疮病例，其黏膜、躯干、四肢（包括伸侧和屈侧）广泛受累，伴有疣状甲沟炎和沟纹舌。临床检查时，我们在黏膜部位发现多个皮肤水疱、糜烂和溃疡。临床、组织病理学以及直接和间接免疫荧光检查结果均符合增殖性天疱疮。患者已完成生育，因此采用静脉注射地塞米松 - 环磷酰胺冲击（DCP）方案治疗，效果极佳。
