Cutaneous mucinosis in mixed connective tissue disease*

Abstract

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

Review of Primary Cutaneous Mucinoses in Nonlupus Connective Tissue Diseases

“…In 2 patients with MCTD, there was an improvement in cutaneous lesions with an increase of azathioprine to 2 mg/ kg/d in 1 patient and the addition of chloroquine 250 mg/d with an increase in prednisone to 20 mg/d in the other patient. 7 Oral isotretinoin was effective in a patient with HIV, 42 and oral thyroxine reportedly failed in a patient. 36 In patients with SLE, antimalarials have been reported to help, especially in conjunction with systemic corticosteroids.…”

“…18 There have been 2 cases of LM associated with mixed connective tissue disease (MCTD). 7 The discrete papular form of LM is limited to the skin and has little or no morbidity. 1 The usual recommendation is to treat conservatively by monitoring for changes and advising the patient that spontaneous resolution is unlikely, although it has been reported.…”

Keratosis pilaris rubra with mucin deposition

“…The presence of mucin is non‐specific but is important to highlight as a feature of KPR because mucin deposition classically raises concern for connective tissue disease, a more sinister diagnosis. Cutaneous lupus erythematosus is most often associated with secondary mucinosis, but mucin deposition has also been reported in other connective tissue diseases including Sjögren syndrome, systemic sclerosis, rheumatoid arthritis, vasculitis, and dermatomyositis . Further, follicular mucin can be seen in a number of benign conditions, including primary follicular mucinosis (of Pinkus) and secondary follicular mucinoses, including eczematous dermatoses, insect bites, polymorphous light eruption, and side effects of certain drugs (imatinib, β‐blockers).…”

Acral papular mucinosis: a new case of this rare entity

“…The latter group includes: certain endocrinopathies (especially thyroid diseases); toxic diseases (toxic oil syndrome); Eosinophilia-Myalgia Syndrome, nephrogenic fibrosing dermopathy; and diffuse connective tissue diseases (such as lupus erythematosus, and cancer). 1 - 3 …”

“…Cutaneous mucinosis is a group of disorders charaterized by an accumulation of mucin or glycosaminoglycan in the skin and its annexes. 3 Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. To date, there have been only 34 reported cases of this entity that strictly fulfilled the diagnostic criteria proposed by Rongioletti and Rebora in 2001.…”