Rather Parvaiz Anwar, Hussain Mohammad, Bagdadi Farhana
Department of Dermatology, Venereology and Leprosy, Government Medical College, Srinagar, Jammu and Kashmir, India.
Department of Medicine, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
Indian J Dermatol. 2014 Jul;59(4):422. doi: 10.4103/0019-5154.135538.
Lichen myxoedematosus (LM), a form of primary cutaneous mucinosis, may present either as localized less severe form called papular mucinosis or diffuse more severe form called scleromyxoedema. The diffuse form is almost always associated with monoclonal gammopathy, whereas localized form is not. We report an atypical case of localized form of LM associated with multiple myeloma in a 66-year-old male, who presented with asymptomatic waxy papular eruption on extremities, which on histopathological examination confirmed the diagnosis of cutaneous mucinosis. After initially being put on steroids and hydroxychloroquine with minimal improvement, patient subsequently presented with encephalopathy and on evaluation revealed hypernatremia, hypercalcemia, hypergammaglobulinemia, reversal of albumin-globulin (A/G) ratio, azotemia, and lytic lesions in skull X-ray. Bone marrow aspiration and biopsy confirmed multiple myeloma. Patient was successfully treated with standard treatment regimen for multiple myeloma with bortezumib and dexamethasone and his skin lesions subsided completely.
黏液水肿性苔藓（LM）是原发性皮肤黏蛋白病的一种形式，可表现为局部症状较轻的丘疹性黏蛋白病，或弥漫性症状较重的硬化性黏液水肿。弥漫性形式几乎总是与单克隆丙种球蛋白病相关，而局部形式则不然。我们报告了一例66岁男性的非典型局部形式LM合并多发性骨髓瘤的病例，该患者四肢出现无症状的蜡样丘疹性皮疹，组织病理学检查确诊为皮肤黏蛋白病。最初使用类固醇和羟氯喹治疗，改善甚微，随后患者出现脑病，评估发现高钠血症、高钙血症、高球蛋白血症、白蛋白 - 球蛋白（A/G）比值倒置、氮质血症，颅骨X线显示溶骨性病变。骨髓穿刺和活检确诊为多发性骨髓瘤。患者接受了硼替佐米和地塞米松治疗多发性骨髓瘤的标准治疗方案，皮肤病变完全消退。
