Amin Hesham M, McLaughlin Peter, Rutherford Cynthia J, Abruzzo Lynne V, Jones Dan
Departments of Hematopathology, The University of Texas-M.D. Anderson Cancer Center, Houston, Texas 77030, U.S.A.
Am J Dermatopathol. 2002 Jun;24(3):241-5. doi: 10.1097/00000372-200206000-00009.
Cutaneous and systemic plasmacytosis is a rare disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. Previous reports have been largely restricted to the Japanese literature. We present the first documented case of cutaneous and systemic plasmacytosis in a patient residing in the United States. This 49-year-old man, who had immigrated from Korea 19 years earlier, developed innumerable persistent pink-to-brown macular lesions over his trunk and face. Initial and repeat skin biopsy specimens revealed dense perivascular and periadnexal infiltrates of mature plasma cells, and polyclonal plasmacytosis noted on two different biopsy specimens of mildly enlarged lymph nodes. Multiple tiny pulmonary nodules were found to be of the same histologic appearance. No evidence of clonal immunoglobulin gene rearrangements or human herpesvirus type 8 infection was noted in these biopsy specimens. Treatment with antibiotics, systemic chemotherapy, and anti-CD20 antibody therapy failed to eradicate these lesions, which have persisted for 6 years. This case demonstrates that cutaneous and systemic plasmacytosis can arise in a patient of Asian ancestry, even many years after emigration to the United States.
皮肤及系统性浆细胞增多症是一种罕见疾病，其特征为广泛分布的斑疹性皮肤疹，由多克隆淋巴细胞浆细胞浸润组成，并伴有不同程度的皮肤外受累。既往报道大多局限于日本文献。我们报告了美国首例有记录的皮肤及系统性浆细胞增多症病例。这名49岁男性19年前从韩国移民而来，其躯干和面部出现了无数持续存在的粉红色至棕色斑疹。初次及重复皮肤活检标本显示成熟浆细胞密集的血管周围和腺周浸润，在轻度肿大的淋巴结的两份不同活检标本中发现多克隆浆细胞增多。发现多个微小肺结节具有相同的组织学表现。这些活检标本中未发现克隆性免疫球蛋白基因重排或8型人类疱疹病毒感染的证据。抗生素、全身化疗及抗CD20抗体治疗均未能根除这些病灶，这些病灶已持续6年。该病例表明，即使在移民到美国多年后，亚洲血统患者也可能发生皮肤及系统性浆细胞增多症。
