【摘要】 一例13岁女孩面部和四肢为主反复出现红斑、丘疹、水疱样皮损9年,皮疹形态与牛痘水疱病相似(hydroa vacciniforme,HV)。在病情急性进展期,伴高热,体温39℃以上。皮肤科检查:面部水肿,面部和四肢较多的萎缩性圆形和椭网形痘疮样瘢痕,散在红斑、丘疹、坏死和结痂;四肢数个水肿性红色斑块,中心有圆形破溃面。组织学上,在真皮的血管和附属器周围,可见大量表达CD8异形淋巴细胞浸润,诊断CD8~+皮肤T细胞淋巴瘤。糖皮质激素和免疫抑制剂可使病情缓解,随诊至今,无系统受累情况。更多还原

【Abstract】 A 13-year-old girl presented with a 9-year history of hydroa vacciniforme(HV)-like eruption,including erythema,papules and vesicles,mainly distributed at the face and extremities.A high fever(>39℃)developed at the progressive stage of this disease.Physical examination showed variously sized,round or oval,atrophic and variola-like scars along with scattered erythematous patches,papules, necrosis and crusts on the face and extremities.The face was edematous,and there were some edematous and erythematous plaques with a necrotic center on the legs and arms.Histological examination revealed a massive infiltration with atypical CD8~+lymphocytes around the vessels and appendages in dermis.A diagnosis of CD8~+cutaneous T-cell lymphoma(CTCL)was made.Glucocorticoid and immunosuppressants were effective in controlling the condition.Up to the time of the writing,there has not been any definite evidence of systemic involvement.更多还原