Leroy D, Lebrun J, Maillard V, Mandard J C, Deschamps P
Ann Dermatol Venereol. 1982;109(6-7):549-55.
A 49-year old woman had an eruption resembling 'dermatite pustuleuse chronique en foyers a progression excentrique' as initially described by Hallopeau. Numberous vegetating lesions occurred over the genitocrural, pubic, umbilical and submammary regions. The finger-nails showed onycholysis and sterile pustules. No bullae were seen at any time. Histopathological study revealed the presence of acantholysis. Direct immunofluorescent microscopy showed IgG deposits in the intercellular epidermal space. The titer of pemphigus antibody was 1/1,600: the results of these immunofluorescence studies on early pustular lesions as well as vegetating lesions support the view that Hallopeau disease belongs to the 'pemphigus group'. The patient was treated first with oral prednisone (80 mg/day). In twelve months of follow-up care, the vegetating lesions disappeared completely, leaving hyperpigmentation. Prompt recurrence of lesions was observed when corticosteroids were discontinued; reinstitution of this therapy was followed by their regression. A difference exits between pemphigus vegetans of Hallopeau and pemphigus vegetans of Neumann: (1) the Hallopeau type begins with pustules as the primary lesions, followed by vegetations, with a lack of bullae and a preference for flexural areas. Immunofluorescence studies will be the deciding factor in separating pemphigus vegetans of Hallopeau from pyoderma vegetans. (2) The Neumann type is a variant of pemphigus vulgaris in which the denuded areas have a tendency to heal with papillomatous formations. It begins and ends with bullae.
一名49岁女性出现了类似Hallopeau最初描述的“慢性灶性脓疱性皮炎伴离心性进展”的皮疹。在腹股沟、耻骨、脐部和乳房下区域出现了许多增殖性损害。指甲表现为甲分离和无菌性脓疱。任何时候均未见到大疱。组织病理学研究显示存在棘层松解。直接免疫荧光显微镜检查显示在表皮细胞间间隙有IgG沉积。天疱疮抗体滴度为1/1600:这些对早期脓疱性损害以及增殖性损害的免疫荧光研究结果支持Hallopeau病属于“天疱疮组”的观点。患者首先接受口服泼尼松(80mg/天)治疗。在12个月的随访中,增殖性损害完全消失,仅留下色素沉着。停用皮质类固醇后观察到损害迅速复发;重新使用该疗法后损害消退。Hallopeau型增殖性天疱疮与Neumann型增殖性天疱疮之间存在差异:(1)Hallopeau型以脓疱作为原发性损害开始,随后出现增殖,无大疱,且好发于屈侧部位。免疫荧光研究将是区分Hallopeau型增殖性天疱疮与增殖性脓皮病的决定性因素。(2)Neumann型是寻常型天疱疮的一种变异型,其中剥脱区域有以乳头瘤样形成愈合的倾向。它以大疱开始和结束。