Granulomatöses Skleromyxödem mit dermato-neurologischem Syndrom – Vorstellung einer seltenen Erkrankung mit schwerer Komplikation

Zusammenfassung

Es wird über eine Patientin mit Skleromyxödem mit monoklonaler Gammopathie berichtet, welche im klinischen Verlauf ein dermato-neurologisches Syndrom entwickelte. Unter Therapie mit intravenösen Immunglobulinen kam es zu einer deutlichen klinischen Befundbesserung. Das dermato-neurologische Syndrom ist definiert als die Kombination Grippe-ähnlicher Symptome, hohem Fieber, tonisch-klonischer Krämpfe und Koma im klinischen Kontext eines Skleromyxödems und ist ein prinzipiell letales Krankheitsbild. Am vorliegenden Fall wird die dringende Notwendigkeit einer engen interdisziplinären Zusammenarbeit deutlich.

Abstract

We report on a female patient with scleromyxedema with monoclonal gammopathy, who developed a dermatoneurological syndrome in the clinical course. Therapy with intravenous immunoglobulins led to a clear clinical improvement. The dermatoneurological syndrome is defined as the combination of flu-like symptoms, high fever, tonic-clonic convulsions and coma in the clinical context of a scleromyxedema and is a lethal clinical picture. In this case, the urgent need for close interdisciplinary cooperation becomes apparent.

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