Massive cutaneous mucinosis associated with systemic lupus erythematosus

Abstract

We describe a 23-year-old Japanese man with systemic lupus erythematosus (SLE) who developed massive cutaneous mucinosis. He was diagnosed with SLE when he was 11 years old. Prednisolone therapy (30 mg/day) was initiated and reduced to 10 mg/day 3 months later; the SLE had been well-controlled with this dose of prednisolone for 12 years. However, infiltrated erythematous plaques developed on the middle-lateral area of his back at 17 years old and progressed to erythematous and elastic soft tumorous masses over 20 cm in diameter at 23 years old. Biopsies of the lesions on the nape revealed massive mucin deposition. Topical injection with hyaluronidase decreased the lesion. This cutaneous mucinosis can be distinguished clinically and histopathologically from papular and nodular mucinosis associated with SLE. The present case might be an unusual clinical variant of cutaneous mucinosis associated with SLE.

Hyaluronidase in the treatment of papular dermal mucinosis: First case reported in North America

“…However, Waldenstrom's Macroglobulinemia, Hodgkin's and non-Hodgkin's lymphomas have all been associated with the disease [1] . Papular mucinosis also has a well-known association with discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) [4][5][6][7][8][9][10][11][12][13] . Up to 80% of cases of papular mucinosis may be associated with SLE.…”

“…Two other reports of successful treatment of papular mucinosis with intralesional hyaluronidase have been described [9] . While its use for papular mucinosis has not been reported since 1995, more recent success is well-documented in the treatment of scleroderma and pretibial myxedema.…”

“…This disease most commonly affects middle-aged adults and has been associated with monoclonal gammopathy, systemic lupus erythematosus and discoid lupus, independently [2][3][4][5][6][7][8][9][10][11][12][13][14] . The report presents the first case of papular mucinosis in the setting of three concurrent disorders: IgG paraproteinemia, systemic lupus erythematosus and discoid lupus erythematosus.…”

Cutaneous mucinoses

“…5 Tumors containing mucin include neurofibromas, neurilemmomas, nerve sheath myxomas, chondroid syringomas, and some basal cell carcinomas. 336,[343][344][345][346][347][348][349][350][351] In these individuals, the deposition occurs in areas free from specific lesions of lupus erythematosus and it produces clinically distinct manifestations. 342,343 Rarely, patients with SLE and systemic sclerosis can present with papulonodules or plaques due to a diffuse mucinous deposition in the skin (Fig.…”

Nonspecific Cutaneous Manifestations of Systemic Lupus Erythematosus

“…Sometimes, flesh-colored papulonodular cutaneous mucin deposition occurs. Rarely, massive mucin deposition has been reported in Japanese patients with SLE (Maruyama et al 1997). The author and other investigators have seen a group of Japanese patients possessing anti-Ro/SSA antibodies who have demonstrated a very peculiar "donut-like" erythematous plaque-like lesion (Nishikawa andProvost 1991, Teramato et al 1989).…”