Rare dermatological entities associated with monoclonal gammopathy: sclerodema, scleromyxedema and IgA pemphigus – overview of 5 cases and therapeutic modalities

Czech version

Authors: Z. Král ¹; M. Krejčí ¹; L. Pour ¹; S. Ševčíková ²; Z. Čermáková ³; Z. Adam ¹
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Ústav patologické fyziologie LF MU a FN Brno ²; Oddělení klinické biochemie FN Brno ³
Published in: Transfuze Hematol. dnes,26, 2020, No. 3, p. 226-235.
Category: Case Report

Overview

Scleroderma adultorum and scleromyxedema are rare scleromucinous diseases that characteristically lead to wooden induration located on the neck and upper trunk. Three types of scleroderma and scleromyxedema can be distinguished according to their association with pre-existing or underlying disease: association with infections (mostly streptococcal infections of the upper respiratory tract), association with monoclonal gammopathy and association with diabetes mellitus. The clinical findings, disease extent and course differ substantially depending on the subtype of scleroderma. Spontaneous regression often occurs in infection-associated scleroderma or scleromyxedema, whereas patients with diabetes or monoclonal gammopathy usually have a chronic progressive course. In cases with monoclonal gammopathy treatment leading to suppression of monoclonal gammopathy may alleviate the dermatologic symptoms. Intravenous immunoglobulins at a dose of 2g/kg can help in cases resistant to chemotherapy. Immunoglobulin (Ig)A pemphigus is a rare disease characterised by a vesiculopustular eruption with intercellular IgA deposition in the epidermis. In this article, we report our experience with 5 cases of rare skin diseases associated with monoclonal gammopathy and present an overview of treatment possibilities.

Keywords:

scleroderma adultorum – scleromyxedema – monoclonal gammopathy – IgA pemphigus

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