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J Cutan Med Surg. 2013 Jul-Aug;17(4):226-32.

Familial pityriasis rubra pilaris: case report and review.

Author information

1
Discipline of Medicine, Faculty of Medicine, Memorial University of Newfoundland, Gander, NL. jmmercer82@gmail.com

Abstract

BACKGROUND:

Pityriasis rubra pilaris (PRP) is a rare dermatosis of unknown etiology. Most cases of PRP are sporadic; however, rare cases of familial PRP have been reported.

OBJECTIVES:

To present a case of PRP inherited in an autosomal dominant (AD) fashion and to evaluate the current literature on familial PRP and formulate a comprehensive, up-to-date summary of this rare condition.

METHODS:

PubMed was used to conduct a search for articles pertaining to familial PRP published through May 2011.

RESULTS:

The first documented case was published in 1910, and 36 subsequent familial cases of PRP have been reported. Familial PRP typically presents very early in childhood, has a gradual onset, and persists throughout life. Given the rarity of this subtype, determining the best therapy has been a challenge. In the pediatric population, a conservative treatment approach, including topical therapy, is frequently used, whereas systemic treatments are reserved for patients with a severe disease that is refractory to therapy.

CONCLUSION:

Rare cases of PRP inherited in an AD fashion have been described and tend to have a chronic clinical course and are treatment refractory. Therefore, the awareness of familial PRP is important for early and accurate diagnosis and administration of appropriate therapy.

PMID:
23815954
DOI:
10.2310/7750.2012.12018
[Indexed for MEDLINE]
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