Castanet J, Lacour J P, Chartier C, Perrin C, Martin T, Grosshans E, Ortonne J P
Serice de Dermatologie, Hôpital de l'Archet II, Nice.
Ann Dermatol Venereol. 1997;124(10):717-20.
Monoclonal gammopathy and edema are features encountered during several diseases, especially systemic capillary leak syndrome. The diagnoses of POEMS syndrome, edematous systemic scleroderma and a fortuitous association may be also discussed. We report the cases of two patients which did not fulfill the criteria for such diagnoses.
Although the 2 cases share some discrepancies, they have also similar and particular features: association of chronic edema, monoclonal gammopathy and profuse and acquired telangiectasias.
The meaning of these cases remains to be clarified. It might be an entity close to the systemic capillary leak syndrome but characterized by the chronicity of edema and by a distinct cutaneous sign, the occurrence of numerous telangiectasias. These cases emphasize that the acquired and profuse telangiectasias belong to the wide range of cutaneous abnormalities which may be associated with monoclonal paraproteinemia.
单克隆丙种球蛋白病和水肿是多种疾病中出现的特征，尤其是系统性毛细血管渗漏综合征。也可能会讨论POEMS综合征、水肿性系统性硬化症及偶然关联的诊断。我们报告了2例不符合此类诊断标准的患者病例。
尽管这2例病例存在一些差异，但它们也有相似且独特的特征：慢性水肿、单克隆丙种球蛋白病与大量后天性毛细血管扩张的关联。
这些病例的意义仍有待阐明。它可能是一种与系统性毛细血管渗漏综合征相近的疾病实体，但以水肿的慢性病程及独特的皮肤表现（大量毛细血管扩张的出现）为特征。这些病例强调后天性大量毛细血管扩张属于可能与单克隆副蛋白血症相关的广泛皮肤异常范畴。
