Hermans C, Goldscheider I, Ruzicka T, Rongioletti F
Department of Dermatology and Allergy, Ludwig- Maximilian University Munich, Munich, Germany.
Department of Medical Science, Unit of Dermatology, University of Cagliari, Cagliari, Italy.
J Eur Acad Dermatol Venereol. 2016 Dec;30(12):2048-2051. doi: 10.1111/jdv.13833. Epub 2016 Jul 16.
Discrete papular lichen myxedematosus (DPLM) is a rare form of localized lichen myxedematosus that presents with skin involvement only and without systemic involvement.
To describe our experience with atypical cases of DPLM associated with monoclonal gammopathy.
Data were collected from patients with clinicopathological evidence of DPLM associated with monoclonal gammopathy who presented to the Department of Dermatology of two tertiary university-affiliated medical centres from 2000 to 2015 and were followed prospectively.
The sample included four patients (three males) with a mean age of 58 years. No clinicopathological differences from typical cases of DPLM were observed, except for the presence of monoclonal gammopathy. The patients were followed up for a mean of 34 months (6-72 months) and no progression to scleromyxedema, multiple myeloma or systemic involvement was observed. No therapy was applied, except for topical tacrolimus or steroids, and the eruptions remained stable.
Our experience indicates an excellent prognosis of DPLM even for atypical cases in spite of the presence of monoclonal gammopathy.
离散性丘疹性黏液性水肿（DPLM）是局限性黏液性水肿的一种罕见形式，仅累及皮肤，无全身受累。
描述我们对与单克隆丙种球蛋白病相关的非典型DPLM病例的经验。
收集2000年至2015年就诊于两所大学附属三级医疗中心皮肤科的有DPLM与单克隆丙种球蛋白病相关的临床病理证据的患者的数据，并进行前瞻性随访。
样本包括4例患者（3例男性），平均年龄58岁。除存在单克隆丙种球蛋白病外，未观察到与典型DPLM病例有临床病理差异。患者平均随访34个月（6 - 72个月），未观察到进展为硬化性黏液性水肿、多发性骨髓瘤或全身受累。除局部使用他克莫司或类固醇外，未应用其他治疗，皮疹保持稳定。
我们的经验表明，即使对于存在单克隆丙种球蛋白病的非典型病例，DPLM的预后也很好。
