Bork K, Böckers M, Pfeifle J
Department of Dermatology, University of Mainz, West Germany.
Arch Dermatol. 1990 Apr;126(4):509-13.
We describe a 62-year-old man with multiple myeloma who developed horny spicules on his face, particularly on his nose. IgG-lambda monoclonal gammopathy was detected, and the serum dysprotein was shown to be a cryoglobulin, which forms a cryogel at low temperatures. Light and electron microscopic and immunohistochemical examinations showed an intercellular precipitation and massive accumulation of the IgG dysprotein and cryoglobulin between the keratinocytes of the upper epidermis and the infundibular epithelium. The follicles were dilated and filled with parakeratotic cells, the protein deposits between them and a rudimentary hair thus resulting in the clinically visible symptoms of horny spicules. The limitation or the predominance of the symptoms in cold-exposed body regions, the morphological identification of the dysprotein deposits as cryoglobulin or cryogel, and the laboratory findings concerning the temperature and pH dependence of the precipitation of the IgG dysprotein reveal that the paraneoplastic horny spicules are a hitherto unknown clinical manifestation of cryoglobulinemia.
我们描述了一名62岁的多发性骨髓瘤男性患者，其面部尤其是鼻子上出现了角质小刺。检测到IgG-λ单克隆丙种球蛋白病，血清异常蛋白显示为冷球蛋白，在低温下形成冷凝胶。光镜、电镜和免疫组化检查显示，在上表皮角质形成细胞和漏斗状上皮之间存在细胞间沉淀以及IgG异常蛋白和冷球蛋白的大量积聚。毛囊扩张，充满不全角化细胞、它们之间的蛋白质沉积物和一根发育不全的毛发，从而导致临床上可见的角质小刺症状。症状在暴露于寒冷的身体部位的局限性或优势性、将异常蛋白沉积物形态学鉴定为冷球蛋白或冷凝胶，以及关于IgG异常蛋白沉淀的温度和pH依赖性的实验室检查结果表明，副肿瘤性角质小刺是冷球蛋白血症一种迄今未知的临床表现。
