Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION

PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians.

OBJECTIVE

To add our experience to increase evidence about PRP.

METHODS

We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP.

RESULTS

PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response.

CONCLUSION

Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.