甲泼尼龙联合阿维A治疗增殖型天疱疮一例

Successful treatment of pemphigus vegetans with acitretin and methylprednisolone： a case report

患者女，55岁。因口腔溃疡，头皮、颈、腋下增殖性斑块4年就诊。皮肤科检查：左舌缘见蚕豆大小的溃疡面，头皮枕部、颈部、双腋下可见肥厚性增生性斑块，表面见少许分泌物，有异味。皮损组织病理检查：角化过度，棘层增生肥厚，基底层上部棘刺松解性水疱形成，疱内有棘刺松解细胞，真皮内可见大量中性粒细胞、淋巴细胞为主的炎细胞浸润，并有中性粒细胞小脓肿形成。直接免疫荧光：表皮棘细胞间IgG（＋）。诊断为增殖型天疱疮。给予静脉滴注甲泼尼龙40mg／d和口服阿维A30mg／d及其他对症治疗。20d后，患者皮损明显好转，渗出减少，斑块变平。

A 55-year-old female patient was referred with a complaint of oral ulcers and vegetative plaques on the scalp, neck and axillae for 4 years. Physical examination revealed bean-sized ulcers at the left rim of the tongue as well as hypertrophic and vegetative plaques with a small amount of exudates and an offensive odor on the scalp, neck and both axillae. Pathological examination showed hyperkeratosis, hyperacanthosis, vesicles caused by acantholysis in the upper part of basal layer, massive inflammatory infiltration of neutrophils and lymphocytes in dennis with neutrophil microabscess. Acantholytic cells were observed in the vesicles. Direct immunofluorescence revealed positive staining for immunoglobulin G （IgG） between epidermal prick cells. The diagnosis of pemphigus vegetans was confirmed based on the above findings. A combination therapy with intravenous methylprednisolone 40 mg once a day and oral acitretin 10 mg twice daily were subsequently initiated along with symptom-oriented approaches. After 20-day treatment, the lesions markedly improved with decreased exudates and flattened plaques.