Familial Pityriasis Rubra Pilaris: Report of a Family and Therapeutic Response to Etanercept

July 2010 | Volume 9 | Issue 7 | Case Reports | 844 | Copyright © July 2010

Marie Vasher MD, Erica Smithberger MD, Mary H. Lien MD, Neil Alan Fenske MD

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis of unknown etiology. The familial subtype is rare and usually presents as type V PRP. It is generally inherited in an autosomal dominant fashion with variable expression. Other forms of inheritance, such as autosomal recessive and X-linked, have also been reported. The use of etanercept in treating resistant forms of PRP is promising given reports of its success in a few cases. Herein, the authors report two cases of PRP arising in a mother and son and review the rare familial subtype of this disease. In addition, a successful therapeutic trial of etanercept was initiated in the mother based on case reports of its efficacy in other patients with PRP.