Lee Joyce Ss, Chiam Lynn, Tan Kong Bing, Salto-Tellez Manuel, Tan Suat Hoon, Ong Beng Hock, Ng See Ket
Department of Dermatology, National Skin Centre, Singapore.
Am J Dermatopathol. 2011 Jul;33(5):498-503. doi: 10.1097/DAD.0b013e3181ea1198.
Cutaneous plasmacytosis is a rare disease entity presenting with multiple extensive red-brown plaques, histopathology showing marked hyperplasia of mature polyclonal plasma cells, and polyclonal hypergammaglobulinemia on serum protein electrophoresis, in the absence of an underlying secondary cause. We report in this article the first case of cutaneous plasmacytosis from Singapore. A 33-year-old Chinese woman presented with mildly pruritic reddish brown papules and plaques over her trunk and arms for 2 years. Physical examination, laboratory investigations, and radiographic examination were negative for systemic involvement and lymphadenopathy. Serum immunoelectrophoresis showed polyclonal hypergammaglobulinemia with immunoglobulin G and immunoglobulin A. Two sets of skin biopsies performed 2 years apart essentially showed similar histopathological findings of a superficial and deep perivascular infiltrate with numerous mature plasma cells and small typical lymphocytes. There were lymphoid follicles with well-formed germinal centers and mantle zones, surrounded by mature lymphocytes. No light chain restriction was present on immunohistochemistry, and polymerase chain reaction for heavy chain gene rearrangement was negative for monoclonality. Despite potent topical corticosteroids and 8 months of phototherapy with narrow band ultraviolet light, there was no improvement. Intralesional triamcinolone injections to a few lesions afforded temporary relief of itch and flattening of lesions.
皮肤浆细胞增多症是一种罕见的疾病实体，表现为多发广泛的红棕色斑块，组织病理学显示成熟多克隆浆细胞显著增生，血清蛋白电泳显示多克隆高球蛋白血症，且无潜在的继发原因。我们在本文中报告了新加坡首例皮肤浆细胞增多症病例。一名33岁中国女性，躯干和手臂出现轻度瘙痒的红棕色丘疹和斑块2年。体格检查、实验室检查及影像学检查均未发现全身受累及淋巴结病。血清免疫电泳显示免疫球蛋白G和免疫球蛋白A多克隆高球蛋白血症。相隔2年进行的两组皮肤活检基本显示出相似的组织病理学表现，即浅层和深层血管周围有大量成熟浆细胞和小的典型淋巴细胞浸润。有形成良好生发中心和套区的淋巴滤泡，周围为成熟淋巴细胞。免疫组化未发现轻链限制，重链基因重排的聚合酶链反应单克隆性为阴性。尽管使用了强效外用糖皮质激素并进行了8个月的窄谱紫外线光疗，但病情无改善。对少数皮损进行皮损内注射曲安奈德可暂时缓解瘙痒并使皮损变平。
