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.

Scleromyxedema (Arndt - Gottron Syndrome) Developing Under Tenofovir Treatment for Hepatitis B: Unique Presentation in a Bulgarian Patient!

“…However, it is possible to observe scleromyxedema without underlying paraproteinemia, as in our patient [3]. A major sign of this disease is the histopathological triad of dermal mucin deposition, fibroblast proliferation, and fibrosis [2]. The clinical condition is characterised by widespread lichenoid papules which coalesce to form generalised plaques that cause extensive thickening and hardening of the skin [2], [3].…”

“…Scleromyxedema or Arndt-Gottron (S-AG) syndrome is a cutaneous mucinosis that mainly affects adults between 30 and 70 years of age and whose aetiology is not fully understood [2]. The disease is defined as a systemic form of Lichen myxedematosus [5].…”

“…Scleromyxedema, a systemic form of lichen myxedematosus (LM) [1], is associated with significant mortality [2], [3], [4]. Interesting in this regard is the association of scleromyxedema with hepatitis virus [5].…”

Rare case of infiltrative cardiomyopathy secondary to scleromyxoedema

“…Scleromyxoedema is a rare systemic condition, with evidence for treatment limited to case series at best. These suggest poor prognosis with cardiac involvement . Previous case reports have not shown infiltrative changes on CMR…”

Successful Coronary Artery Bypass Grafting in a Patient With Uncontrolled Scleromyxedema

“…It is characterized by diffuse cutaneous and systemic mucinosis with paraproteinemia. The definitive pathophysiology of why this occurs in the setting of a paraproteinemia remains unclear [1][2]. The diagnosis of scleromyxedema is based upon the recognition of the following clinicopathologic criteria: generalized, papular, and sclerodermoid eruption; microscopic triad, including mucin deposition, fibrosis, and fibroblast proliferation, or, less frequently, an interstitial granulomatous-like pattern; monoclonal gammopathy; absence of thyroid disorder.…”