2016International Medical Case Reports JournalOpen access

Atypical scleromyxedema presenting with cutaneous and cardiovascular manifestations

David A. Kandiah, Sue Ann Teh

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Abstract

Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy.

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What this paper is about

Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy.

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Available abstract

Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy.

Key concepts: Medicine, Mucinosis, Dermatology, Myxedema, Monoclonal gammopathy, Paraproteinemias, Pathology, Monoclonal

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