Case for diagnosis. Lichen myxedematosus

Abstract

Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.

Lichen myxedematosus: a rare group of cutaneous mucinosis

“…The scalp and mucosa are generally not affected. 9 Papules are commonly arranged in a linear array ( Figure 2) and the surrounding skin has a sclerodermoid appearance. There is no significant predominance by gender, and it is most common in adults between the fifth and sixth decades of life.…”

Papular mucinosis (papular lichen myxedematosus): Clinical and histopathological evaluation

“…To the best of our knowledge, this is the first study on localized lichen myxedematosus (papular mucinosis) showing ten cases with different variants of the condition, with previous studies being either case reports [5,[11][12][13]18] or a study with one type of localized cutaneous mucinosis [24].…”

“…Scleromyxedema is a rare cutaneous disorder characterized by dispersed eruption of hard, waxy, dome-shaped or flat-topped papules and nodules 2 -3 mm in size that may amalgamate to form plaques and that involve the head, neck, trunk, and extremities. The scalp and mucosa are usually uninvolved [5]. Papules are generally arranged linearly and the neighboring skin exhibits a sclerodermoid manifestation.…”

Metabolic and Nutritional Disorders Relevant in Dermatology

“…• Definition: Lichen myxoedematosus, also known as scleromyxedema, is a rare type of papular mucinosis with a chronic and progressive course (Rebellato et al 2016) manifested by the deposition of mucin in the forms of papules, nodules, and plaques leading to a waxy appearance of the skin. Increased serum levels of IL-1, TNF-alpha, and TGF-beta have been reported in this disorder, which results in stimulation of glycosaminoglycan synthesis and proliferation of fibroblast.…”