Pityriasis rubra pilaris - a retrospective single center analysis over eight years

Summary Background: Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis. We evaluated evaluate co-morbidities, complications, and outcome of treatment regimens. Patients and Methods: This is a retrospective study at an academic teaching hospital. We analyzed all patients with the definite diagnosis of PRP seen since 2001. Epidemiologic data, co-morbidities, response to and course during treatment were investigated. Results: We identified 10 PRP-patients (6 men, 4 women), mean age 56.4 years, with type I (n = 9) and type IV (n = 1). Three patients had internal co-morbidities (atrial fibrillation with cardiac insufficiency, dilated cardiomyopathy, diabetes mellitus). Two patients needed psychiatric treatment because of depression. PRP caused ectropium (2 ×), diffuse effluvium (1 ×), and stenosis of the outer ear canal (1 ×). We did not observe a spontaneous remission. Among 9 patients with PRP type I, five were treated with acitretin (two of them as Re-PUVA), and two with methotrexate (in one patient combined with fumaric acids). Systemic corticosteroids were not effective. One patient was treated with infliximab i.v., 5 mg/kg body weight. Starting with the first application, inflammatory activity decreased and erythema got paler. The treatment was well tolerated. Conclusions: PRP type I is a severe, chronic inflammatory dermatosis responding hesitantly to classic systemic therapies. Tumor necrosis factor-α antagonists are an effective treatment option for difficult cases.