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: Marie Vasher, Erica Smithberger, Mary H Lien, Neil Alan Fenske
• Journal: Journal of drugs in dermatology : JDD (J Drugs Dermatol) Vol. 9 Issue 7 Pg. 844-50 (Jul 2010) ISSN: 1545-9616 [Print] United States
• PMID: 20677542 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin G
• Receptors, Tumor Necrosis Factor
• Tumor Necrosis Factor-alpha
• Etanercept

Topics

• Adolescent
• Diagnosis, Differential
• Etanercept
• Female
• Humans
• Immunoglobulin G (therapeutic use)
• Male
• Middle Aged
• Pityriasis Rubra Pilaris (drug therapy, genetics, pathology)
• Receptors, Tumor Necrosis Factor (therapeutic use)
• Tumor Necrosis Factor-alpha (antagonists & inhibitors)