eMedicine Specialties > Dermatology > Papulosquamous Diseases

Pityriasis Rubra Pilaris: Differential Diagnoses & Workup

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

Differential Diagnoses

Cutaneous T-Cell Lymphoma
Erythroderma (Generalized Exfoliative Dermatitis)
Erythrokeratodermia Variabilis
Psoriasis, Plaque

Workup

Laboratory Studies

  • No specific laboratory tests are available to confirm the diagnosis of pityriasis rubra pilaris (PRP). The diagnosis is usually made on the basis of a correlation between clinical findings and histologic findings.12
  • Perform tests for electrolyte abnormalities, hypoalbuminemia, secondary bacterial infection in the skin, and possible sepsis.

Procedures

  • Perform a biopsy.

Histologic Findings

Histologic features are not pathognomonic, but they are useful to rule out other possible papulosquamous and erythrodermic disorders. Features on light microscopy include hyperkeratosis with alternating orthokeratosis and parakeratosis forming a checkerboard pattern in the stratum corneum, focal or confluent hypergranulosis, follicular plugging with perifollicular parakeratosis forming a shoulder effect, thick suprapapillary plates, broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic perivascular infiltration. Acantholysis has been reported as an additional histologic finding in pityriasis rubra pilaris.13 Acantholysis may be restricted to adnexal epithelium. The presence of acantholysis, hypergranulosis, follicular plugging, and the absence of dilated capillaries and epidermal pustulation may help distinguish pityriasis rubra pilaris from psoriasis.14

Features on electron microscopy include a decreased number of keratin filaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipidlike vacuoles, large numbers of lamellar granules, and a focal split in the basal lamina at the dermoepidermal junction.

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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