Pityriasis Rubra Pilaris 

  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 26, 2010
 

Background

Pityriasis rubra pilaris (PRP) was first described in 1828 by Tarral and was named by Besnier in 1889. It is a chronic papulosquamous disorder of unknown etiology characterized by reddish orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. The disease may progress to erythroderma with distinct areas of uninvolved skin, the so-called islands of sparing.

Griffiths divided pityriasis rubra pilaris into 5 categories: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, and atypical juvenile type.[1, 2] More recently, an HIV-associated type has been added to this classification system.[3, 4, 5, 6]

A few reports have also described pityriasis rubra pilaris associated with underlying malignancy.[7]

Other eMedicine pityriasis articles include Pityriasis Alba, Pityriasis Lichenoides, Pityriasis Rosea, and Pityriasis Rotunda.

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Pathophysiology

The etiology is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern; however, most cases are sporadic.[8] One hypothesis is that pityriasis rubra pilaris may be related to an abnormal immune response to an antigenic trigger. Case reports have described pityriasis rubra pilaris occurring after streptococcal infections.[9]

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Epidemiology

Frequency

United States

The incidence of pityriasis rubra pilaris has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics.

Mortality/Morbidity

Patients with pityriasis rubra pilaris can have painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may be present. However, most of the morbidity associated with pityriasis rubra pilaris is associated with the erythroderma (see Complications).

Race

Persons of any race can be affected.

Sex

Pityriasis rubra pilaris occurs equally among men and women.[10]

Age

The familial form of pityriasis rubra pilaris typically begins in early childhood and has an autosomal dominant inheritance pattern.

The acquired form of pityriasis rubra pilaris has a bimodal age distribution, with peaks in the first and fifth decades of life, but it can begin at any age.

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Contributor Information and Disclosures
Author

Philip D Shenefelt, MD, MS  Associate Professor, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine; Past Chief, Section of Dermatology, James A Haley Veteran Affairs Medical Center

Philip D Shenefelt, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Physician Executives, American Contact Dermatitis Society, American Medical Association, American Society of Clinical Hypnosis, Florida Medical Association, Noah Worcester Dermatological Society, and Society for Clinical and Experimental Hypnosis

Disclosure: Nothing to disclose.

Specialty Editor Board

Mark G Lebwohl, MD  Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Amgen/Pfizer Honoraria Consulting; Centocor/Janssen Honoraria Consulting; DermiPsor Honoraria Consulting; GlaxoSmithKline Honoraria Consulting; HelixBioMedix Honoraria Consulting; Novartis Honoraria Consulting; Ranbaxy Lectures; Can-Fite Biopharma Honoraria Consulting; DermaGenoma Honoraria Consulting; Biosynexus Honoraria Consulting

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  2. Griffiths WA. Pityriasis rubra pilaris: the problem of its classification. J Am Acad Dermatol. Jan 1992;26(1):140-2. [Medline].

  3. Auffret N, Quint L, Domart P, Dubertret L, Lecam JY, Binet O. Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. J Am Acad Dermatol. Aug 1992;27(2 Pt 1):260-1. [Medline].

  4. Blauvelt A, Nahass GT, Pardo RJ, Kerdel FA. Pityriasis rubra pilaris and HIV infection. J Am Acad Dermatol. May 1991;24(5 Pt 1):703-5. [Medline].

  5. Martin AG, Weaver CC, Cockerell CJ, Berger TG. Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne. Br J Dermatol. Jun 1992;126(6):617-20. [Medline].

  6. Miralles ES, Nunez M, De Las Heras ME, Perez B, Moreno R, Ledo A. Pityriasis rubra pilaris and human immunodeficiency virus infection. Br J Dermatol. Dec 1995;133(6):990-3. [Medline].

  7. Kurzydlo AM, Gillespie R. Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma. Australas J Dermatol. May 2004;45(2):130-2. [Medline].

  8. Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P. Familial pityriasis rubra pilaris. Arch Dermatol. Apr 1995;131(4):448-53. [Medline].

  9. Mohrenschlager M, Abeck D. Further clinical evidence for involvement of bacterial superantigens in juvenile pityriasis rubra pilaris (PRP): report of two new cases. Pediatr Dermatol. Nov-Dec 2002;19(6):569. [Medline].

  10. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol. Sep 2002;47(3):386-9. [Medline].

  11. Martinez Calixto LE, Suresh L, Matsumura E, Aguirre A, Radfar L. Oral pityriasis rubra pilaris. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. May 2006;101(5):604-7. [Medline].

  12. Abbott RA, Griffiths WA. Pityriasis rubra pilaris type 1 spontaneously resolving after 20 years. Clin Exper Dermatol. 2009;34:378-379.

  13. Cohen PR, Prystowsky JH. Pityriasis rubra pilaris: a review of diagnosis and treatment. J Am Acad Dermatol. May 1989;20(5 Pt 1):801-7. [Medline].

  14. Howe K, Foresman P, Griffin T, Johnson W. Pityriasis rubra pilaris with acantholysis. J Cutan Pathol. Jun 1996;23(3):270-4. [Medline].

  15. Magro CM, Crowson AN. The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. J Cutan Pathol. Aug 1997;24(7):416-24. [Medline].

  16. Van de Kerkhof PC, Steijlen PM. Topical treatment of pityriasis rubra pilaris with calcipotriol. Br J Dermatol. May 1994;130(5):675-8. [Medline].

  17. Karimian-Teherani D, Parissa M, Tanew A. Response of juvenile circumscribed pityriasis rubra pilaris to topical tazarotene treatment. Pediatr Dermatol. Jan-Feb 2008;25(1):125-6. [Medline].

  18. Vergilis-Kalner IJ, Mann DJ, Wasserman J, Petronic-Rosic V, Toukas, MM. Pityriasis rubra pilaris sensitive to narrow band-ultraviolet B light therapy. J Drugs Dermatol. 2009;8:270-273.

  19. Haenssle HA, Bertsch HP, Emmert S, Wolf C, Zutt M. Extracorporeal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris. Clin Exp Dermatol. May 2004;29(3):244-6. [Medline].

  20. Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. Int J Dermatol. Apr 2006;45(4):438-46. [Medline].

  21. Cox V, Lesesky EB, Garcia BD, O'Grady TC. Treatment of juvenile pityriasis rubra pilaris with etanercept. J Am Acad Dermatol. Nov 2008;59(5 Suppl):S113-4. [Medline].

  22. Liao WC, Mutasim DF. Infliximab for the treatment of adult-onset pityriasis rubra pilaris. Arch Dermatol. Apr 2005;141(4):423-5. [Medline].

  23. Manoharan S, White S, Gumparthy K. Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab. Australas J Dermatol. May 2006;47(2):124-9. [Medline].

  24. Muller H, Gattringer C, Zelger B, Hopfl R, Eisendle K. Infliximab monotherapy as first-line treatment for adult-onset pityriasis rubra pilaris: case report and review of the literature on biologic therapy. J Am Acad Dermatol. Nov 2008;59(5 Suppl):S65-70. [Medline].

  25. Dicken CH. Treatment of classic pityriasis rubra pilaris. J Am Acad Dermatol. Dec 1994;31(6):997-9. [Medline].

  26. Clayton BD, Jorizzo JL, Hitchcock MG, et al. Adult pityriasis rubra pilaris: a 10-year case series. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):959-64. [Medline].

  27. Wetzig T, Sticherling M. Juvenile pityriasis rubra pilaris: successful treatment with ciclosporin. Br J Dermatol. Jul 2003;149(1):202-3. [Medline].

  28. Borok M, Lowe NJ. Pityriasis rubra pilaris. Further observations of systemic retinoid therapy. J Am Acad Dermatol. May 1990;22(5 Pt 1):792-5. [Medline].

  29. Walling HW, Swick BL. Pitryiasis rubra pilaris responding rapidly to adalimumab. Arch Dermatol. 2009;145:99-101.

  30. Greene R. PRP support group. Dermatol Nurs. Feb 2006;18(1):28. [Medline].

  31. Griffiths WA. Pityriasis rubra pilaris--an historical approach. 2. Clinical features. Clin Exp Dermatol. Mar 1976;1(1):37-50. [Medline].

 
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Reddish orange plaques on the trunk.
Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.
Plantar keratoderma with an orange hue on the soles.
Palmar keratoderma with an orange hue on the palms.
 
 
 
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