国内首报落叶型天疱疮转变为大疱性类天疱疮1例

Pemphigus foliaceus converting into bullous pemphigoid:the first case reported in China

患者女,58岁,头颈胸背起皮疹伴痒痛20d,于2010年2月入院,皮疹表现为红斑基础上浅表糜烂,尼氏征阳性。组织病理检查示颗粒细胞层及棘细胞层上方裂隙,并可见游离角化不良细胞,直接免疫荧光(DIF)示棘细胞间IgG和C3沉积,诊断为落叶型天疱疮,予以曲安西龙24mg/d,雷公藤多苷片60 mg/d,烟酰胺300 mg/d和四环素2 g/d治疗,皮疹逐渐消退。2017年6月,患者头颈、胸背及手部起皮疹伴瘙痒15 d再次入院,皮疹为红斑基础上紧张水疱,尼氏征阴性。组织病理检查见表皮下水疱,疱内及真皮浅层见嗜酸性粒细胞及淋巴细胞浸润,DIF示IgG和C3沿基底膜带沉积,ELISA检查示BP180抗体阳性,Dsg1和Dsg3抗体阴性,诊断为大疱性类天疱疮,予以曲安西龙12 mg/d、烟酰胺300 mg/d和四环素2 g/d口服,皮损逐渐消退。

A 58-year-old female patient presented with painful and itchy skin lesions on the head,neck,chest and back for 20 days,and was admitted to the hospital in February 2010.The skin lesions manifested as superficial erosions on an erythematous base with positive Nikolsky's sign.Histopathological examination showed fissures above the granular and spinous cell layers,and scattered dyskeratotic cells.Direct immunofluorescence(DIF)assay revealed IgG and C3 deposits between spinous cells.The patient was initially diagnosed with pemphigus foliaceus.After the treatment with triamcinolone(24 mg/d),tripterygium glycosides(60 mg/d),nicotinamide(300 mg/d)and tetracycline(2 g/d),skin lesions gradually subsided.In June 2017,the patient was readmitted due to itchy skin lesions on the head,neck,chest,back and hands for 15 days,which manifested as tense bullae on an erythematous base with negative Nikolsky's sign.Histopathological examination showed subepidermal blisters and infiltration of eosinophils and lymphocytes in the blisters and superficial dermis,DIF assay revealed the deposition of IgG and C3 along the basement membrane zone,and enzyme-linked immunosorbent assay showed positive results for anti-BP 180 antibody but negative results for anti-Dsgl and Dsg3 antibodies.Then,a diagnosis of bullous pemphigoid was confirmed.The patient was treated with oral triamcinolone(12 mg/d),nicotinamide(300 mg/d)and tetracycline(2 g/d),and the lesions rapidly subsided.