Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

Abstract

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

Periorbital mucinosis: A variant of cutaneous lupus erythematosus?

“…Distinctive (primary) lesions of cutaneous mucinosis in association with dermatomyositis are exceptional, but have been reported. [23][24][25][26] These cases consistently presented in association with proximal muscle weakness and elevated levels of creatine kinase. 23 In our cases, the absence of the classic manifestations of dermatomyositis, supported Those patients who have been reported with cutaneous lupus erythematosus involving the eyelids usually have a rapid response to antimalarial treatment.…”

Scleroderma-like cutaneous syndromes

“…While scleromyxedema is not associated with Raynaud's phenomenon, prominent visceral organ involvement is common. Inflammatory myopathy and esophageal dysmotility may be seen, leading in some cases to possible misdiagnosis as systemic sclerosis [42,43]. Monoclonal gammopathy is frequently detected in the serum and may be associated with multiple myeloma [44].…”

Scleromyxedema without paraproteinemia

“…Histopathology is characterized by deposits of mucin in the upper and middle reticular dermis, with increased collagen and fibroblast proliferation with irregular distribution . Given the difficulty involved in diagnosing this disease, diagnostic criteria were defined in 2001 . These include a generalized papular and sclerodermoid rash, mucin deposits, fibroblast proliferation and fibrosis, monoclonal gammopathy, and the absence of thyroid disease .…”

“…8 These include a generalized papular and sclerodermoid rash, mucin deposits, fibroblast proliferation and fibrosis, monoclonal gammopathy, and the absence of thyroid disease. 8 The pathogenesis of this disease remains uncertain; however, the high prevalence of monoclonal gammopathy, predominantly of immunoglobulin G (IgG), which presents in up to 80% of patients, suggests a possible immune response of B cells to antigenic mucin deposits in the dermis. 3,5,[9][10][11][12] Although a slight plasmacytosis has been detected in bone marrow, monoclonal gammopathy progresses to multiple myeloma in only 10% of cases.…”