Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept
Vasher, M.; Smithberger, E.; Lien, M.H.; Fenske, N.Alan.
Journal of Drugs in Dermatology Jdd 9(7): 844-850
2010
ISSN/ISBN: 1545-9616 PMID: 20677542 Document Number: 12637
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.
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