种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

Hydroa Vacciniforme-like T Cell Lymphoma Complicated with Primary Cutaneous CD30-positive Large Cell Lymphoma:A Case Report

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。

A 17-year-old girl presented with polymorphous lesions including erythemas, papules, papulovesicles, vesicles, necrosis, pitted scars on face, neck, upper back, wraist, buttocks, extremities as well as fever and itching for 15 years, and tumors on extremities for 2 years. The viral serological diagnosis showed EBV IgG positivity, but EBV IgM negativity. Histopathology of the tumor showed diffuse and dense infiltrate of lymphoid ceils with anasplastic nuclei in the middle and low potion of the dermis as well as the subcutis. The tumor cells were positive for CD3 and CD43,1arge cells in infiltration for CD30 and 80% tumor cells for Ki-67. Histopathology of the vesicular lesion showed reticular degeneration and vesicles in the epidermis. A moderate to dense infiltrate of lymphoid cells,with an angiocentric and periappendageal pattern in the dermis and subcutis,was observed. Small numbers of eosinophils were also present in the dermal infiltrates. Some large cells in the infiltrates had a significant heteromorphism. The heteromorphic cells were positive for CD3 ,CD43 and Ki-67 ,but negative for CD30. A diagnosis of hydroa vacciniforme-like T cell lymphoma complicated with primary cutaneous CD30-positive large cell lymphoma was made. Then the patient was transferred into the oncological department for chemotherapy.