Dermatomyositis with Prominent Mucinous Skin Change

Abstract

Although there is occasionally remarkable accumulation of mucin in the skin lesion of dermatomyositis, no special attention has been paid in regard to mechanisms underlying it. We carried out histochemical and biochemical studies on materials obtained from one case of dermatomyositis, who showed the presence of abundant mucinous material stainable with alcian blue between the collagen bundles throughout the lesional dermis. The major component of the ground substance was identified to be hyaluronic acid. The activity of N-acetyl-β-glucosaminidase, exoglycosidase that degrades hyaluronic acid in serum of the patient, was equivalent to that of controls. However, heparan sulfate was greatly increased in the urine of the patient in comparison with that of the normal controls in which the major components were chondroitin 4-sulfate and chondroitin 6-sulfate. We think that the increase of hyaluronic acid in the lesional dermis is due to an excess production by the fíbroblasts under some immunological stimulation, although its relation to the increased heparan sulfate excretion into the urine of the patient is obscure.

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

“…Cutaneous mucinosis is subdivided into primary and secondary types; in primary, mucin deposition is the primary histologic feature and secondary, where mucin deposition is an additional finding to a primary clinicopathologic setting. Cutaneous manifestations of mucin can be focal or diffuse and are described as dermal or epidermal (follicular) [9, 14]. The pathophysiology of increased mucin deposition in connective tissue diseases is not completely understood and it is a rare finding.…”

Low-Dose Methotrexate as an Adjunctive Therapy with Surgery for Ectropion Complicating Dermatomyositis

“…The ectropion was presumed to be secondary to chronic inflammation, poikilodermatous change and mucinous infiltration of the eyelids. Cutaneous mucin deposits are a common finding in dermatomyositis; how ever, they only rarely produce clinically de tectable changes in the skin [4][5][6][7].…”