CAJ | 학술논문

种痘样水疱病样皮肤T细胞淋巴瘤是近年来被逐渐认识到的一种与EB病毒相关的少见淋巴瘤,多见于儿童和青少年,主要累及面部和四肢,表现为丘疹、丘疱疹和水疱,随后出现坏死、溃疡、结痂,最后留下痘疮样瘢痕.本病临床表现独特,确诊主要依靠组织病理检查及免疫组化.目前尚无统一有效的治疗方案,报道的多数病例采用化疗,但疗效不一.
충두양수포병양피부T세포림파류시근년래피축점인식도적일충여EB병독상관적소견림파류,다견우인동화청소년,주요루급면부화사지,표현위구진、구포진화수포,수후출현배사、궤양、결가,최후류하두창양반흔.본병림상표현독특,학진주요의고조직병리검사급면역조화.목전상무통일유효적치료방안,보도적다수병례채용화료,단료효불일.
Hydroa vacciniforme-like cutaneous T-cell lymphoma is a rare subtype of cutaneous T cell lymphoma identified in the past few years.The association between this condition and Epstein Barr virus (EBV) infection has been gradually recognized.It mainly affects children and teenagers with the involvement of face and extremities.The clinical manifestations of this entity are distinct,and lesions usually begin with papules,papulovesicles,vesicles,subsequently develop into necrosis,ulcer and crusts,and heal with varioliform scarring.The diagnosis is mainly based on histopathology and immunohistochemistry.At present there is no uniform and effective treatment.Most reported cases were managed by chemotherapy,but the efficacy varied.