Son Young-Min, Kang Hong-Kyu, Yun Jeong-Hwan, Roh Joo-Young, Lee Jong-Rok
Department of Dermatology, Gachon University of Medicine and Science, Gil Hospital, Incheon, Korea.
Ann Dermatol. 2011 Dec;23(Suppl 3):S310-3. doi: 10.5021/ad.2011.23.S3.S310. Epub 2011 Dec 27.
Pemphigus vegetans is a rare variant of pemphigus vulgaris and is characterized by vegetating lesions in the inguinal folds and mouth and by the presence of autoantibodies against desmoglein 3. Two clinical subtypes of pemphigus vegetans exist, which are initially characterized by flaccid bullae and erosions (the Neumann subtype) or pustules (the Hallopeau subtype). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery of the lesions. Oral administration of corticosteroids alone does not always induce disease remission in patients with pemphigus vegetans. We report here on a 63-year-old woman with pemphigs vegetans. She had a 2-year history of vegetating, papillomatous plaques on the inguinal folds and erosions of the oral mucosa. The enzyme-linked immunosorbent assay was positive for anti-desmoglein 3, but it was negative for anti-desmoglein 1. She was initially treated with systemic steroid, but no improvement was observed. The patient was then successfully treated with a combination of systemic steroid and dapsone with a good clinical response.
增殖性天疱疮是寻常型天疱疮的一种罕见变异型，其特征为腹股沟皱襞和口腔出现增殖性损害，且存在抗桥粒芯糖蛋白3的自身抗体。增殖性天疱疮有两种临床亚型，最初分别表现为松弛性水疱和糜烂（诺伊曼亚型）或脓疱（阿洛波亚型）。两种亚型随后都会发展为色素沉着的增殖性斑块，伴有脓疱以及损害周边的肥厚性肉芽组织。对于增殖性天疱疮患者，仅口服皮质类固醇并不总能诱导疾病缓解。我们在此报告一名63岁的增殖性天疱疮女性患者。她有2年腹股沟皱襞处出现增殖性乳头状斑块及口腔黏膜糜烂的病史。酶联免疫吸附试验抗桥粒芯糖蛋白3呈阳性，但抗桥粒芯糖蛋白1呈阴性。她最初接受全身用类固醇治疗，但未见改善。该患者随后采用全身用类固醇与氨苯砜联合治疗，获得了良好的临床反应，治疗成功。
