Dermatomyositis and Lichen Myxedematosus

Abstract

The association of lichen myxedematosus and dermatomyositis is uncommon. The patient in this study developed cutaneous mucinosis several years before the myositis. Histological and histochemical studies confirmed the diagnosis of a mucinosis and myositis, and showed that the fibroblast plays a significant role in the production of the skin lesions.

Diffuse Cutaneous Mucinosis in Dermatomyositis: A Case Report and Review of the Literature

“…Activation of proinflammatory cytokines and chemokines leads to the migration of lymphoid cells to the perimysial and endomysial spaces; complement activation leads to the formation and deposition of membranolytic attack complexes onto endomysial capillaries. The result is microangiopathy and necrosis of endothelial cells leading to perivascular inflammation, muscle ischemia, and muscle fiber destruction [8, 13]. …”

“…The pathophysiology of increased mucin deposition in connective tissue diseases is not completely understood and it is a rare finding. It is postulated that substances circulating in the serum, such as immunoglobulins, autoantibodies, or cytokines, stimulate glycosaminoglycan synthesis by fibroblasts leading to the production of mucin and its deposition in the skin [8, 12]. Pandya et al linked the increased level of serum autoantibody titres with an increase in mucin lesions in patients with SLE [11, 15].…”

“…Clinically evident forms of mucinosis have been described in hypothyroidism, thyrotoxicosis, scleromyxedema associated with monoclonal gammopathies, scleredema related to diabetes, and lichen myxedematosus. Cases of secondary cutaneous mucinosis have been described in systemic lupus erythematosus, systemic sclerosis, and dermatomyositis, albeit infrequently [2–8]. We present a case of dermatomyositis with evidence of diffuse cutaneous mucinosis in a patient recently treated for nonsmall cell lung cancer (NSCLC) without evidence of recurrence.…”

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

“…Scleromyxedema can be associated both with systemic involvement because the disease itself can include arthritis, neurological manifestations or myositis 2–5 and, more rarely, with other diseases such as multiple myeloma, non‐Hodgkin lymphoma or amyloidosis. Dermatomyositis (DM) has only exceptionally been described in association with scleromyxedema 6,7 …”

Dermatomyositis with Prominent Mucinous Skin Change

“…DM [4,5], SLE [4], pa raproteinemia [6]). Janis and Winkelmann [4] noted the presence of mucin deposits in the specimens of 29 of 55 cases of DM; these findings were seen not only in the inflamma tory poikilodermatous lesion but also in the nonspecific inflammatory skin.…”