Miest Rachel Y N, Comfere Nneka I, Dispenzieri Angela, Lohse Christine M, el-Azhary Rokea A
Department of Dermatology, Mayo Clinic, RochesterDepartment of Laboratory Medicine and Pathology, Mayo Clinic, RochesterDivision of Hematology, Mayo Clinic, RochesterDivision of Clinical Biochemistry and Immunology, Mayo Clinic, RochesterDivision of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA.
Int J Dermatol. 2013 Nov;52(11):1349-56. doi: 10.1111/j.1365-4632.2012.05648.x. Epub 2013 Apr 4.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia.
From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. We assessed prevalence of skin findings and associations between dermatologic and other characteristic disease findings.
Of the 107 patients, 96 (90%) had a recognized cutaneous manifestation. Hyperpigmentation and hemangioma were most common (47%), followed by hypertrichosis (38%). Vascular skin changes--acrocyanosis (34%), Raynaud phenomenon (20%), hyperemia/erythema (20%), flushing (16%), or rubor (11%)--occurred in 62%; white nails, sclerodermoid changes, and clubbing occurred in 30%, 26%, and 6%, respectively. Mean number of skin findings per patient was 2.9 (median, 3.0; range, 0-7). Presence of cutaneous manifestation was associated with abnormal pulmonary function tests (P < 0.001); immunoglobulin G gammopathy was associated with hyperpigmentation and hypertrichosis. No other significant associations were seen.
The high prevalence of skin findings (90%) shows the value of dermatologic evaluation in diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Our data indicate new associations between skin findings and other disease characteristics.
多神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征是一种与浆细胞发育异常相关的罕见多系统副肿瘤性疾病。
从我们机构的异常蛋白血症数据库中，筛选出2000年1月1日至2009年10月1日期间符合多神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征标准的107例患者。回顾病历以了解诊断时记录的综合征特征。我们评估了皮肤表现的患病率以及皮肤病学与其他特征性疾病表现之间的关联。
107例患者中，96例（90%）有公认的皮肤表现。色素沉着过度和血管瘤最为常见（47%），其次是多毛症（38%）。血管性皮肤改变——手足发绀（34%）、雷诺现象（20%）、充血/红斑（20%）、潮红（16%）或发红（11%）——发生率为62%；白甲、硬皮病样改变和杵状指分别发生率为30%、26%和6%。每位患者的皮肤表现平均数量为2.9（中位数为3.0；范围为0 - 7）。皮肤表现的存在与肺功能测试异常相关（P < 0.001）；免疫球蛋白G型丙种球蛋白病与色素沉着过度和多毛症相关。未发现其他显著关联。
皮肤表现的高患病率（90%）表明皮肤病学评估在多神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征诊断中的价值。我们的数据表明皮肤表现与其他疾病特征之间有新的关联。
