Pityriasis Rubra Pilaris Clinical Presentation

  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 20, 2012
 

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 the images below). The lesions may expand and coalesce to cover the entire body.

Plantar keratoderma with an orange hue on the solePlantar keratoderma with an orange hue on the soles. Palmar keratoderma with an orange hue on the palmsPalmar keratoderma with an orange hue on the palms.
Next

Physical

Physical findings can be grouped by skin, nails, mucous membranes, and eyes, as follows:

  • 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 the images below). 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 the images below). This pattern may be referred to as nutmeg grater papules. 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. Reddish orange plaques on the trunk. Reddish orange plaques on the trunk. Follicular hyperkeratosis seen on the dorsal aspecFollicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.
  • 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

The Griffiths classification includes types I through IV, as follows:

  • 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. One reported case resolved spontaneously after 20 years.[12]
  • 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.
Previous
Next

Causes

The etiology is unknown.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Philip D Shenefelt, MD, MS  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; GlaxoSmithKline Honoraria Consulting; Novartis Honoraria Consulting; Ranbaxy Honoraria Lectures; Pfizer Honoraria Consulting; BioLineRX, Ltd. Honoraria Consulting; Celgene Corporation Consulting; Clinuvel None Investigator; Eli Lilly & Co. None Investigator; Genentech 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; Celgene Honoraria 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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Margaret H. Rinker, MD, to the development and writing of this article.

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. 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. Garcovich S, Di Giampetruzzi AR, Antonelli G, Garcovich A, Didona B. Treatment of refractory adult-onset pityriasis rubra pilaris with TNF-alpha antagonists: a case series. J Eur Acad Dermatol Venereol. Aug 2010;24(8):881-4. [Medline].

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

  31. Wohlrab J, Kreft B. Treatment of pityriasis rubra pilaris with ustekinumab. Br J Dermatol. Sep 2010;163(3):655-6. [Medline].

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

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

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.