亲毛囊性蕈样肉芽肿一例

Folliculotropic mycosis fungoides: a case report

患者,男,28岁。全身瘙痒11年,头颈、躯干及双上肢红色丘疹、斑块伴嗜酸粒细胞增多5年,脱发2年。外周嗜酸粒细胞计数11 088/μL。组织病理示:毛囊周围中等量单一核细胞浸润,以不典型淋巴细胞为主,并见散在嗜酸粒细胞。免疫组化染色示浸润细胞主要是CD4+细胞。TCR基因重排示TCRδ、TCRγ均阳性。诊断:亲毛囊性蕈样肉芽肿。给予重组人干扰素α-2b肌注,阿维A、甲氨喋呤、泼尼松等口服,糖皮质激素软膏外用。治疗7周后皮损好转。

A 28-year-old male presented with pruritus all over his body for 11 years,red papules and plaques on head,neck,trunk and arms with peripheral eosinophilia for 5 years,and alopecia for 2 years.Peripheral blood eosinophilic cell count was 11088/μL.Histopathological findings showed a mixed perifollicular infiltration which was composed mainly of irregular-shaped atypical lymphocytes and scattered eosinophils.Immunohistochemical staining indicated that the majority of infiltrated lymphocytes were CD4+lymphocytes.TCR gene rearrangement showed TCRδand TCRγwere positive.The diagnosis of folliculotropic mycosis fungoides was made.The lesions were improved after 7 weeks treatment of intramuscular injections of recombinant interferonα-2b,oral acitretin,methotrexate,prednisone and topical corticosteroids cream.