Efficacy of intravenous immunoglobulin in the treatment of scleromyxoedema

Annales de dermatologie et de vénéréologie
L SillardJ-P Ortonne

Abstract

Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG). A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease. The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurre...Continue Reading

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Citations

Jul 19, 2012·Zeitschrift für Rheumatologie·A KreuterK Möllenhoff
May 3, 2020·International Journal of Dermatology·Roger HaberMaria El Gemayel
Dec 30, 2020·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Jochen H O Hoffmann, Alexander H Enk

Dec 30, 2020·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Jochen H O Hoffmann, Alexander H Enk

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