中文摘要：

报告1例5-氨基酮戊酸光动力疗法（5-Aminolevulinic Acid Photodynamic Therapy, ALA-PDT）治疗慢性家族性良性天疱疮（Hailey-Hailey Disease,HHD）。患者男,31岁,因双侧腹股沟及肛周浸渍糜烂10年就诊,否认家族遗传史。皮肤科情况：右腹股沟及肛周红斑、浸渍、糜烂,部分皲裂,少量渗出,可见色素沉着,未见皮肤明显增厚。皮损组织病理检查：表皮局部角化不全,表皮突下延,棘层增厚,全层棘刺松解,呈“倒塌砖墙样”改变,真皮浅层小血管周围少量炎细胞浸润。符合慢性家族性良性天疱疮（HHD）诊断。使用5-氨基酮戊酸光动力疗法（ALA-PDT）治疗3次,皮损基本消退,随访6个月未复发。

英文摘要：

A case of familial benign chronic pemphigus（Hailey-Hailey disease, HHD）treated by 5-Aminolevulinic Acid Photodynamic Therapy（ALA-PDT）is reported. A 31-year-old man presented with macerated and crusted erosions on bilateral inguinal and perianal region for more than 10 years. There was no family history of HHD. Dermatological examination showed erythematous, macerated and crusted erosions, cracked and few of exudation in the right inguinal and perianal region. Development of postinflammatory hyperpigmentation was seen. There has no obvious thicking of the skin. Histopathological examination revealed focal parakeratosis, extended trochanterellus, acanthosis and acantholysis throughout the spinous layer, which have been likened to a ＂dilapidated brick wall＂. The inflammatory cells infiltration distribution in the superficial of the dermis small blood vessels. The diagnosis of HHD was made. Significant improvement was achieved in our patient after three treatments of ALA-PDT. During a follow-up period of 6 months, the patient had no relapse in the treated lesions.