Scleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin

Abstract

Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg) appears to be the therapy of choice for scleromyxedema. Treatment experiences in atypical manifestations with mucinous nodules are limited to sporadic reports. We report the case of male patient with atypical scleromyxedema without underlying paraproteinemia, presenting with generalized papular and sclerodermoid skin eruption and multiple nodular mucinous lesions on the fingers and face as well as on the eyelids, and associated systemic symptoms. Complete regression of all cutaneous lesions and extracutaneous symptoms with sustained remission was achieved by combined treatment with thalidomide and IVIg.

Atypical scleromyxedema with prominent nodular lesions associated with immune thrombocytopenia: An unusual presentation

“…3 Vemurafenib treatment of patients with metastatic melanoma carrying the BRAFV600E mutation results in partial or complete tumor regression in the majority of patients and increases overall survival time. 4,5 Common adverse events during vemurafenib therapy include photosensitivity, fatigue, rash, and arthralgia. 4,5 Furthermore, approximately one third of the patients develop epithelial tumors of the skin such as warts, squamous cell carcinomas, and keratoacanthomas.…”

“…4,5 Common adverse events during vemurafenib therapy include photosensitivity, fatigue, rash, and arthralgia. 4,5 Furthermore, approximately one third of the patients develop epithelial tumors of the skin such as warts, squamous cell carcinomas, and keratoacanthomas. 1,4,5 Hereby, we wish to report a case of lupus erythematosuselike skin eruption after vemurafenib therapy in a 70-year-old woman with stage IIIc progressive metastatic melanoma (pT4bN3M0).…”

“…4,5 Furthermore, approximately one third of the patients develop epithelial tumors of the skin such as warts, squamous cell carcinomas, and keratoacanthomas. 1,4,5 Hereby, we wish to report a case of lupus erythematosuselike skin eruption after vemurafenib therapy in a 70-year-old woman with stage IIIc progressive metastatic melanoma (pT4bN3M0). After excision of a 8-mm thick primary tumor on the left upper aspect of her back and lymph node dissection of the left axilla (7 metastatic of 19 lymph nodes), the tumor reoccurred within 26 months.…”

“…3 Interestingly, the recently reported cases, with a prominent nodular eruption, presented in absence of underlying paraproteinemia. 3,4 Associated hematologic diseases in SM include Waldenstr€ om macroglobulinemia, Hodgkin lymphoma, and non-Hodgkin lymphoma, particularly after chemotherapy with melphalan. 1 To our knowledge, immune thrombocytopenia has not been reported previously in SM.…”

Scleromyxedema without Paraproteinemia: Treatment with Thalidomide and Prednisolone

“…There are two main divisions of lichen myxedematosus: (a) generalized papular and sclerodermoid form (scleromyxedema) with systemic, even lethal, manifestation, and (b) a localized form without a demonstrable paraprotein, which runs a more benign course [2]. Diagnostic criteria include generalized papules and sclerodermoid eruption, mucin deposition and fibroblast proliferation, monoclonal gammopathy, and absence of thyroid disease [3]. …”