Scleromyxedema: clinical diagnosis and autopsy findings

Abstract

Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.

Figure 1

Papulareruption associated with erythema on the back and neck

Figure 2

Coalescing normochromic papules, some in linear arrangement, and sclerodermiform surrounding skin

Figure 3

Erythematous areas interspersed with pallor and evidence of proximal weakness

Figure 4

Positive colloi - dal iron staining, identifying mucopolysaccharides between the collagen bands (400x)

Figure 5

Dense superficial perivascular inflammatory infiltrates (HE, 100x)