title: Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept.
abstract: 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.
authors.affiliation: Department of Dermatology and Cutaneous Surgery, University of South Florida,Tampa, FL 33612, USA.
authors.forename: Marie
authors.initials: M
authors.lastname: Vasher
authors.forename: Erica
authors.initials: E
authors.lastname: Smithberger
authors.forename: Mary H
authors.initials: MH
authors.lastname: Lien
authors.forename: Neil Alan
authors.initials: NA
authors.lastname: Fenske
mesh_terms: D000293:Adolescent; D003937:Diagnosis, Differential; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010916:Pityriasis Rubra Pilaris / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept