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eISSN: 2084-9893
ISSN: 0033-2526
Dermatology Review/Przegląd Dermatologiczny
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without publication fees
6/2010
vol. 97
 
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abstract:
Original paper

Two cases of scleromyxedema

Agnieszka Terlikowska-Brzósko
,
Bartłomiej Kwiek
,
Olga Glińska
,
Anna Kozłowska
,
Maria Błaszczyk
,
Jadwiga Dwilewicz-Trojaczek
,
Wojciech Sachs

Przegl Dermatol 2010, 97, 378–385
Online publish date: 2011/01/03
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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.
keywords:

scleromyxedema, lichen myxedematosus, monoclonal gammopathy
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