Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: case report

BMC Dermatology
Frank BreuckmannAlexander Kreuter

Abstract

Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible. We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and Alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1+ and anti-mastcell-tryptase+ cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins. Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therap...Continue Reading

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Citations

Oct 30, 2009·American Journal of Clinical Pathology·Thilo GambichlerGisela Schieren
May 3, 2020·International Journal of Dermatology·Roger HaberMaria El Gemayel
Sep 11, 2007·Journal of Dermatological Science·Thilo GambichlerAlexander Kreuter

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