Lortscher David N, Amundson Stan A, Pittelkow Mark R
University of California, San Diego, San Diego, California, USA.
Dermatol Online J. 2010 Dec 15;16(12):4.
A 71-year-old woman presented with exquisitely tender mucosal erosions, a diffuse polymorphous eruption, and night sweats. Workup revealed multiple myeloma with a monoclonal IgG-kappa paraprotein in the serum. Her severe oral involvement was suggestive of paraneoplastic pemphigus, but direct and indirect immunofluorescence tests were negative. A skin biopsy showed spongiosis and a sparse perivascular lymphocytic infiltrate, with occasional CD8-positive lymphocytes in the epidermis. Her lesions improved with intravenous immune globulin. Immunohistochemical staining on the formalin-fixed biopsy specimen was strongly positive for IgG and IgG-kappa in an epidermal "chicken-wire" pattern, but negative for IgG-lambda. Her pulmonary tissue stained negative for IgG-kappa, suggesting clinical relevance of the myeloma paraprotein in her epidermis. To our knowledge, this is the first report of a multiple myeloma patient with such an eruption.
一名71岁女性出现极度疼痛的黏膜糜烂、弥漫性多形性皮疹和盗汗。检查发现患有多发性骨髓瘤,血清中有单克隆IgG-κ副蛋白。她严重的口腔受累提示副肿瘤性天疱疮,但直接和间接免疫荧光试验均为阴性。皮肤活检显示海绵形成和稀疏的血管周围淋巴细胞浸润,表皮偶尔有CD8阳性淋巴细胞。静脉注射免疫球蛋白后她的病变有所改善。福尔马林固定活检标本的免疫组化染色显示,IgG和IgG-κ呈表皮“筛状”模式强阳性,但IgG-λ阴性。她的肺组织IgG-κ染色阴性,提示骨髓瘤副蛋白在其表皮中的临床相关性。据我们所知,这是首例有多发性骨髓瘤伴此类皮疹的患者报告。