Jansen T, Messer G, Meurer M, Plewig G
Klinik für Dermatologie und Allergologie der Ruhr-Universität Bochum, Gudrunstrasse 56, 44791 Bochum.
Hautarzt. 2001 Jun;52(6):504-9. doi: 10.1007/s001050000088.
Pemphigus vegetans is a rare variant of pemphigus which is mainly localized in the intertriginous areas (Neumann type, Hallopeau type) or primarily involves the mucous membranes (pyostomatitis vegetans). A 18-year-old patient with erosive stomatitis developed a vegetating plaque with papillomatous and verruciform features in her left axilla. Histopathological examination of the axilla revealed papillomatosis and acanthosis as well as suprabasal clefting with acantholytic cells. By direct immunofluorescent examination, deposits of immunoglobulin IgG and complement (C3) were found in the intercellular space of the epidermis. Serological examination by indirect immunofluorescent techniques was indicative of pemphigus autoantibodies at a titer of 1:40. This case report demonstrates that the classical differentiation of pemphigus vegetans in two types, Neumann type and Hallopeau type, is mainly of historical importance, because both entities may represent variants of the same disease. In addition, other disorders such as IgA pemphigus may also present with vegetating plaques in intertriginous sites.
增殖性天疱疮是天疱疮的一种罕见变异型,主要局限于皮肤褶皱部位(诺伊曼型、哈洛佩型)或主要累及黏膜(增殖性脓性口炎)。一名患有糜烂性口炎的18岁患者在左腋窝出现了具有乳头瘤样和疣状特征的增殖性斑块。对腋窝进行组织病理学检查发现有乳头瘤病、棘层肥厚以及伴有棘层松解细胞的基底上裂隙。通过直接免疫荧光检查,在表皮细胞间间隙发现了免疫球蛋白IgG和补体(C3)沉积。采用间接免疫荧光技术进行血清学检查显示天疱疮自身抗体滴度为1:40。本病例报告表明,增殖性天疱疮分为诺伊曼型和哈洛佩型这两种经典类型,主要具有历史意义,因为这两种类型可能代表同一种疾病的不同变异型。此外,其他疾病如IgA天疱疮也可能在皮肤褶皱部位出现增殖性斑块。