Pityriasis rubra pilaris – eine seltene entzündliche Dermatose mit vielen Facetten

Zusammenfassung

Die Pityriasis rubra pilaris (PRP) ist eine seltene entzündliche, papulosquamöse Dermatose, die in 6 verschiedenen Manifestationsformen (Subtypen) sowohl bei Kindern als auch bei Erwachsenen mit großer Variabilität auftreten kann. Bei allen Subtypen sind in den Anfangsstadien follikulär gebundene, keratotische Papeln zu beobachten, die nach variabler Latenz zu unterschiedlich großen, gut begrenzten Plaques mit krankheitstypischem, orange-rotem Farbton konfluieren. Dabei finden sich bei den ausgedehnteren Verlaufsformen charakteristischerweise innerhalb der Plaques Inseln gesunder Haut (nappes claires). Nicht selten entsteht eine palmare und plantare, wechselnd stark ausgeprägte Hyperkeratose. Die PRP bietet ein breites klinisches Spektrum, das von einem lokalisierten Befall der Extremitäten bis hin zu erythrodermatischen Formen reicht, die mitunter bereits im Säuglingsalter beginnen können.

Die vorliegende Arbeit gibt eine Übersicht über den aktuellen Kenntnisstand dieser bislang wenig verstandenen Dermatose.

Abstract

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease. There are 6 distinct subtypes with striking clinical variation in both children and adults. Basic elements noted across all subtypes include follicular keratotic papules that evolve to distinct, well-demarcated, scaling plaques of various sizes with typical reddish-orange hue. More generalized subtypes tend to show intervening areas of unaffected skin, known as “islands of sparing” (nappes claires), which represent a characteristic feature. PRP shows a broad spectrum of presentations ranging from mild manifestations confined to extremities to severe disease developing into erythroderma.

The present review provides an overview of the current state of knowledge of this hitherto poorly understood dermatosis.

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