毛囊黏蛋白病

A case of follicular mucinosis

报告1例毛囊黏蛋白病。患者女,32岁。头部、面部及背部红斑伴脱发半年。头部可见3个甲盖大的局限性脱发区,并伴局部脱屑,面部及背部可见大小不等的红斑伴脱屑。皮损组织病理检查示表皮轻度角化过度伴角化不全,棘层增厚,皮突不规则向下伸长;真皮血管及毛囊周围轻度淋巴细胞浸润,毛囊明显变性,局部形成空腔,有黏液样物质沉积。黏蛋白染色(+),抗酸染色(-)。免疫组化:扁豆凝集素(LCA)(+)、CD3(灶性+)、CD20(灶性+)、CD4(-)及CD75(-)。T细胞抗原受体(TCR)γ基因重排呈多克隆特性。诊断:毛囊黏蛋白病。治疗:予口服硒脂糖、维生素E及咪唑斯汀,外用地塞米松及氧化锌对症治疗。治疗4个月后皮疹消退,近8年患者随访无复发。

A case of follicular mucinosis is reported. A 32-year-old woman presented with erythema on her head, face and back, and alopecia appeared on her scalp for half a year. Physical examination showed 3 nail-sized localized alopecia areas on her head with desquamation, and different size erythema on her face and back with mild desquamation. Histopathological examination showed slight hyperkeratosis and parakeratosis in the epidermis with thickened spinous layer and irregular elonga- tion of the rete ridges, and mild lymphocytic infiltration around blood vessel and hair follicles in the dermis. The hair follicles were obvious degenerative and formation of localized cavities filled with mucous-like materials. Mucin staining（＋）, acidfast staining（-）. Immunohistochemical examination showed LCA（＋）, focal CD3 and CD20 （＋）, CD4（-）, and CD75（-）. Using nested PCR with two pairs of primers, T-cell receptor gene rearrangement study showed polyclonal features. Diagnosis： follicular mucinosis. Treatment： Polysaccharide selenate capsules, vitamin E capsules, mizolastine tablets were prescribed. Dexamethasone cream and zinc oxide ointment were topically used. Four months later, the lesions disappeared without recurrence up to date.