Generalized papular and sclerodermoid eruption: scleromyxedema

• Published in: Indian journal of dermatology, venereology, and leprology Vol. 76; no. 5; p. 592
• Main Authors: Serdar, Zehra Asiran, Altunay, Ilknur Kivanc, Yasar, Sirin Pekcan, Erfan, Gamze Türker, Gunes, Pembegül
• Format: Journal Article
• Language: English
• Published: India Medknow Publications and Media Pvt. Ltd 01-09-2010; Scientific Scholar
• Subjects: Biopsy; Bone marrow; Case studies; Diagnosis; Drug therapy; Facies; Health aspects; Humans; Immunoglobulin G; Isotretinoin; Lichenoid Eruptions - pathology; Light; Male; Middle Aged; Myxedema; Pathogenesis; Photodynamic therapy; Physiological aspects; Risk factors; Scleromyxedema - pathology; Skin - pathology; India
• ISSN: 0378-6323; 0973-3922; 1998-3611
• DOI: 10.4103/0378-6323.69096

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.