Generalized papular and sclerodermoid eruption: scleromyxedema.
Scleromyxedema (SM) is a rare chronic progressive and highly intractable cutaneous disease with unknown etiology, affecting both genders equally between 30 and 50 years. The disease is characterized with mucin deposits in the skin and/or other organs. In fact it is a clinicopathological subset of lichen myxedematosus (LM) according to a new classification. Sclerodermiform plaques and lichenoid papules are characteristic cutaneous lesions. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Generalized form is quite difficult to treat and may even be fatal. Herein, we present a male patient with typical features of generalized papular and sclerodermoid LM variety and with benign outcome by isotretinoin.
Serdar
Zehra Asiran
ZA
Haydarpasa Training, Dermatology and Venerology Clinic, Istanbul, Turkey.
Altunay
Ilknur Kivanc
IK
Yasar
Sirin Pekcan
SP
Erfan
Gamze Türker
GT
Gunes
Pembegül
P
D001706:Biopsy; D019066:Facies; D006801:Humans; D017512:Lichenoid Eruptions / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D053718:Scleromyxedema / Q000473:pathology*; D012867:Skin / Q000473:pathology*