Introduction . Scleromyxedema is a chronic disease numbered among mucinoses. Diagnostic criteria are: characteristic scleroderma-like skin lesions, monoclonal gammopathy without thyroid gland dysfunction, and, in histopathological examination, fibroblast proliferation and mucin deposits in the extracellular matrix.

Objective . To present 2 cases of scleromyxedema with different types of skin lesions predominating.

Case report . Two cases of scleromyxedema with different clinical manifestations are presented. In the first patient a scleroderma-like lesion predominated, while in the second one papular eruption was mainly observed. Each patient had individualized therapy; however, neither prednisone combined with chlorambucil and pulses with cyclophosphamide and prednisone in the first patient nor melphalan pulses in the second one were sufficiently effective.

Conclusions . Regardless of the clinical presentation, management of scleromyxedema can be challenging because of limited efficacy, safety or accessibility of different recommended treatment modalities.