eMedicine Specialties > Dermatology > Papulosquamous Diseases

Pityriasis Rubra Pilaris

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
Contributor Information and Disclosures

Updated: May 22, 2009

Introduction

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.

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

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.

Clinical

History

  • The familial form of pityriasis rubra pilaris has a gradual onset, whereas the acquired form has an acute onset.
  • The disease typically spreads in a craniocaudal direction.
    • Patients first notice redness and scales on the face and the scalp.
    • This is often followed by redness and thickening of the palms and the soles (see Media Files 3-4).


Plantar keratoderma with an orange hue on the sol...

Plantar keratoderma with an orange hue on the soles.

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Plantar keratoderma with an orange hue on the sol...

Plantar keratoderma with an orange hue on the soles.



Palmar keratoderma with an orange hue on the palm...

Palmar keratoderma with an orange hue on the palms.

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Palmar keratoderma with an orange hue on the palm...

Palmar keratoderma with an orange hue on the palms.

  • The lesions may expand and coalesce to cover the entire body.

Physical

  • Skin
    • Pityriasis rubra pilaris is characterized by orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body (see Media File 1).


Reddish orange plaques on the trunk.

Reddish orange plaques on the trunk.

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Reddish orange plaques on the trunk.

Reddish orange plaques on the trunk.

    • Often, areas of uninvolved skin, referred to as islands of sparing, are present.
    • Follicular hyperkeratosis is commonly seen on the dorsal aspects of the proximal phalanges, the elbows, and the wrists (see Media File 2). This pattern may be referred to as nutmeg grater papules.


Follicular hyperkeratosis seen on the dorsal aspe...

Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.

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Follicular hyperkeratosis seen on the dorsal aspe...

Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.

    • Palmoplantar keratoderma occurs in most patients and tends to have an orange hue. Painful fissures may develop in patients with palmoplantar keratoderma.
    • Pruritus, although not a major symptom, may occur in the early stages of the disease.
  • Nails
    • Nail changes include distal yellow-brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages.
    • Nail pitting is not typical.
  • Mucous membranes: Patients may complain of pain and irritation in the mouth. Mucous membrane changes include a diffuse whitish appearance of the buccal mucosa, lacy whitish plaques, grayish-white papules and plaques, erythema, or possible erosions.11
  • Eyes: Patients with extensive disease may develop ectropion. Patients have also reported blurred vision and dryness.
  • Griffiths classification
    • Type I is classic adult pityriasis rubra pilaris. This is the most common form of pityriasis rubra pilaris, accounting for more than 50% of all cases of pityriasis rubra pilaris. Onset is acute, and the features are classic, including erythroderma with islands of sparing, palmoplantar keratoderma, and follicular hyperkeratosis. This type of pityriasis rubra pilaris has the best prognosis. Reportedly, about 80% of patients have remission in an average of 3 years.
    • Type II is atypical adult pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. It is characterized by ichthyosiform lesions, areas of eczematous change, alopecia, and long duration (often 20 y or more.)
    • Type III is classic juvenile pityriasis rubra pilaris. This form accounts for about 10% of all cases of pityriasis rubra pilaris. It is very similar to type I; however, its onset is within the first 2 years of life. Remission can occur sooner than with type I, within an average of 1 year.
    • Type IV is circumscribed juvenile pityriasis rubra pilaris. This form accounts for about 25% of all cases of pityriasis rubra pilaris. It occurs in prepubertal children and is characterized by sharply demarcated areas of follicular hyperkeratosis and erythema of the knees and the elbows. The long-term outcome is unclear, with some reports of improvement in the late teenaged years. This form of pityriasis rubra pilaris rarely progresses.
    • Type V is atypical juvenile pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. Most cases of familial pityriasis rubra pilaris belong to this group. It has an early onset and runs a chronic course. It is characterized by prominent follicular hyperkeratosis, sclerodermalike changes on the palms and the soles, and infrequent erythema.
    • Type VI is HIV-associated pityriasis rubra pilaris. Patients with HIV may have nodulocystic and pustular acneiform lesions. Elongated follicular plugs or lichen spinulosus–type lesions have also been reported to be present. Patients' conditions tend to be resistant to standard treatments, but they may respond to antiretroviral therapies.

Causes

The etiology is unknown.

More on Pityriasis Rubra Pilaris

Overview: Pityriasis Rubra Pilaris
Differential Diagnoses & Workup: Pityriasis Rubra Pilaris
Treatment & Medication: Pityriasis Rubra Pilaris
Follow-up: Pityriasis Rubra Pilaris
Multimedia: Pityriasis Rubra Pilaris
References
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References

  1. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. Mar 1980;5(1):105-12. [Medline].

  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. 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].

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

  14. 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].

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

  16. 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].

  17. 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].

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

  19. 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].

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

  21. 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].

  22. 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].

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

  24. 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].

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

  26. 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].

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

  28. 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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Further Reading

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Keywords

pityriasis rubra pilaris, PRP, papulosquamous disorder, palmoplantar keratoderma, keratotic follicular papules, erythroderma

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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.

Medical Editor

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: Abbott Laboratories Honoraria Consulting; Actelion Honoraria Consulting; Amgen Honoraria Consulting; Astellas Honoraria Consulting; Centocor Honoraria Consulting; DermiPsor Honoraria Consulting; Galderma  Consulting; Genentech Honoraria Consulting; Helix BioMedix Honoraria Consulting; Medicis Honoraria Investigator

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, 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 Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

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