Purpura pigmentosa progressiva

Zusammenfassung

Die Purpura pigmentosa progressiva (PPP) ist eine benigne, häufig chronisch rezidivierende Dermatose, die sich vor allem symmetrisch an den unteren Extremitäten, in ausgeprägten Fällen auch am Rumpf und den oberen Extremitäten, manifestiert. Auch einmalige Krankheitsverläufe sind beschrieben. Klinisch äußert sich die Purpura pigmentosa progressiva in der Akutphase in Form von meist asymptomatischen petechialen Einblutungen, in ihrem Verlauf mit bräunlich-orangenen Maculae. In einigen Fällen wird von einem geringen Juckreiz berichtet. Meist führt der ästhetische Aspekt zum Arztbesuch. Es werden in der Literatur verschiedene Ursachen diskutiert, der genaue Pathomechanismus ist jedoch unklar. Eine zugrundeliegende Systemerkrankung liegt nicht vor. Histologisch zeigen sich Erythrozytenextravasate, perivaskuläre Lymphozyteninfiltrate und Hämosiderinablagerungen ohne Zeichen einer Vaskulitis. Differenzialdiagnostisch kommen vor allem eine leukozytoklastische Vaskulitis oder Gerinnungsstörungen in Betracht. Therapeutisch empfiehlt sich die Gabe von Vitamin C in Kombination mit Rutosid.

Summary

Progressive pigmented purpuric dermatosis is a benign, often chronically recurring disease. It affects symmetrically the lower extremities, and in pronounced cases, also the trunk and the upper extremities. Also unique events are described. The classical clinical presentation of progressive pigmented purpuric dermatosis includes asymptomatic petechial haemorrhages in the acute phase and brownish-orange macular lesions in the later course of the disease. In some cases a mild itching can be an associated symptom. Patients affected by this condition seek medical help mostly due to aesthetic concerns. Various causes for this condition are discussed in the literature, but the exact underlying pathophysiological mechanism remains unclear. An underlying systemic condition however does not lead to the disease. Histology reveals extravasation of erythrocytes, perivascular lymphocytic infiltrate and hae mosiderin deposits with no signs of vasculitis. Leukocytoclastic vasculitis and coagulation disorders should be considered as differential diagnosis. Therapeutically vitamin C in combination with rutosid is recommended.

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Schlüsselwörter

Purpura pigmentosa progressiva - Dermatose

Keywords

Purpura pigmentosa progressiva - dermatosis

• Literatur

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