Scleromyxedema.

Abstract

A 52-year-old man presented to our outpatient clinic with a 6-month history of generalized asymptomatic eruptions. Physical examination revealed multiple 0.1–0.3-cm firm, waxy, and closely spaced papules with partial confluence on the dorsal hands, forearms, scalp, face, neck, and upper back (Fig. 1, Panels A-1 and A-2). On the dorsal part of the proximal interphalangeal joints, a central depression was surrounded by an elevated rim, which presented a donut-like appearance (Fig. 1, Panel A-1). The skin of the glabella, upper back, and proximal arms was thickened and indurated with deep, longitudinal furrows, presenting typical characteristics of a ‘leonine face’ (Fig. 1, Panel A-3) and ‘Shar-Pei sign’ (Fig. 1, Panel A-4). Skin biopsy revealed scleromyxedema, which is a sclerotic variant of lichen or popular mucinosis in which lichenoid papules and scleroderma-like features are both present [1]. It is a rare mucinous deposition disorder that shares clinical features with scleroderma but has important distinguishing features in clinical presentation and major organ complications that should be recognized [2]. Laboratory tests of the patient revealed normal thyroid function. Serum protein immunoelectrophoresis and immunofixation revealed IgG monoclonal gammopathy. Six months after monthly cyclic treatment of 2 g/kg intravenous immunoglobulin, the skin eruptions improved considerably (Fig. 1, Panels B-1–B-4).