Ichthyosiformer Morphenwandel einer erythrodermen Mycosis fungoides unter extrakorporaler Photopherese-Therapie – klinisch relevant?

Zusammenfassung

Die Mycosis fungoides (MF) tritt klassischerweise in drei Stadien (Patch-, Plaque- und Tumorstadium) auf und ist ein den Dermatologen äußerst geläufiges Krankheitsbild. Zahlreiche morphologische Varianten der MF sind bekannt, von denen drei als separate Varianten in der WHO/EORTC-Klassifikation 2005 aufgeführt werden (follikulotrope MF, pagetoide Retikulose und die sog. „granulomatous slack skin“). Erythroderme Varianten der MF sind von einem Sézary-Syndrom abzugrenzen, für welches definierte Diagnosekriterien existieren. Selten erst wurde über einen ichthyosiformen Subtyp der MF berichtet. Wir stellen den Verlauf einer erythrodermen MF vor, welche unter einer extrakorporalen Photopheresetherapie einen ichthyosiformen Morphenwandel zeigte, diskutieren sowohl klinische als auch histologische Merkmale und stellen pathogenetische Hypothesen zur Diskussion.

Abstract

Mycosis fungoides (MF) typically manifests in three classical clinical stages (patch, plaque and tumor form) and its clinical presentation is well known for dermatologists and dermatopathologists. A great variety of morphologic variants are known and three distinct entities have been named in the WHO/EORTC classification system in 2005: folliculotropic MF, pagetoid reticulosis and granulomatous slack skin. Erythrodermic forms are known and have to be differentiated from a Sézary syndrome. Reports of an ichthyosiform MF are rare. We present the case of an erythrodermic variant of MF with development of ichthyosiform eruptions under extracorporal photopheresis. Clinical and dermatopathological aspects as well as pathogenetic hypothesis are discussed.

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