Pityriasis Rubra Pilaris

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Pityriasis Rubra Pilaris

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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, 8]

Other Medscape pityriasis articles include Dermatologic Manifestations of Pityriasis Alba, Pityriasis Lichenoides, Pityriasis Rosea, and Pityriasis Rotunda.

The etiology is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern. Type V pityriasis rubra pilaris has been linked to mutations in the gene, CARD. [9, 10, 11] Most cases of pityriasis rubra pilaris are sporadic, however. [12] 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. [13]

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

Persons of any race can be affected.

Pityriasis rubra pilaris occurs equally among men and women. [14]

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.

Each type of pityriasis rubra pilaris has its own prognosis. In general, the familial form of the disease may be persistent throughout life, and the acquired form of the disease may resolve spontaneously within 1-3 years. 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).

The prpAlliance (http://prpalliance.com/) is a nonprofit, patient advocacy organization. Their Web site is currently offline, but it directs the reader to the PRP Community on RareConnect (see https://www.rareconnect.org/en/community/pityriasis-rubra-pilaris). Additionally, there is a PRP Facebook Support Group, founded in 2013, for patients and caregivers.

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

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. 1992 Aug. 27(2 Pt 1):260-1. [Medline].

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

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. 1992 Jun. 126(6):617-20. [Medline].

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. 1995 Dec. 133(6):990-3. [Medline].

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

Remedios IM, Jensen JD, Beckum K, McKay K, Kissel R. Paraneoplastic pityriasis rubra pilaris as the presenting manifestation of metastatic squamous cell carcinoma. J Drugs Dermatol. 2014 May. 13(5):610-2. [Medline].

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

Fuchs-Telem D, Sarig O, van Steensel MA, et al. Familial Pityriasis Rubra Pilaris Is Caused by Mutations in CARD14. Am J Hum Genet. 2012 Jul 13. 91(1):163-70.

Takeichi T, Sugiura K, Nomura T, Sakamoto T, Ogawa Y, Oiso N, et al. Pityriasis Rubra Pilaris Type V as an Autoinflammatory Disease by CARD14 Mutations. JAMA Dermatol. 2017 Jan 1. 153 (1):66-70. [Medline].

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

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. 2002 Nov-Dec. 19(6):569. [Medline].

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

Mohamed M, Belhadjali H, Hammedi F, Meriem CB, Zili J. Pityriasis rubra pilaris occurring after vaccination with diphtheria-pertussis-tetanus and oral poliovirus vaccines. Indian J Dermatol Venereol Leprol. 2015 Nov-Dec. 81 (6):618-20. [Medline].

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

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

Martín Callizo C, Molinero Caturla J, Sánchez Sánchez J, Penín Mosquera RM. Scarring Alopecia in Classic Adult Type I Pityriasis Rubra Pilaris. Actas Dermosifiliogr. 2014 Jul 8. [Medline].

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

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

Avitan-Hersh E, Bergman R. The Incidence of Acantholysis in Pityriasis Rubra Pilaris-Histopathological Study Using Multiple-Step Sections and Clinicopathologic Correlations. Am J Dermatopathol. 2015 Oct. 37 (10):755-8. [Medline].

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

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

Lwin SM, Hsu CK, Liu L, Huang HY, Levell NJ, McGrath JA. Beneficial effect of ustekinumab in familial pityriasis rubra pilaris with a new missense mutation in CARD14. Br J Dermatol. 2017 Mar 16. [Medline].

Feldmeyer L, Mylonas A, Demaria O, Mennella A, Yawalkar N, Laffitte E, et al. Interleukin 23-Helper T Cell 17 Axis as a Treatment Target for Pityriasis Rubra Pilaris. JAMA Dermatol. 2017 Apr 1. 153 (4):304-308. [Medline].

Napolitano M, Abeni D, Didona B. Biologics for pityriasis rubra pilaris treatment: a review of the literature. J Am Acad Dermatol. 2018 Mar 30. [Medline].

Maloney NJ, Hisaw LD, Worswick S. Type I pityriasis rubra pilaris treated with tumor necrosis factor inhibitors, ustekinumab, or secukinumab: a systematic review. J Am Acad Dermatol. 2018 Mar 5. [Medline].

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.

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

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

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

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

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. 2010 Aug. 24(8):881-4. [Medline].

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

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

Eytan O, Sarig O, Sprecher E, van Steensel MA. Clinical response to ustekinumab in familial pityriasis rubra pilaris caused by a novel mutation in CARD14. Br J Dermatol. 2014 Aug. 171(2):420-2. [Medline].

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

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

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

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. 2008 Nov. 59(5 Suppl):S65-70. [Medline].

Ruiz Villaverde R, Sánchez Cano D. Successful treatment of type 1 pityriasis rubra pilaris with ustekinumab therapy. Eur J Dermatol. 2010 Sep-Oct;. 20(5):630-1.

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

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

Koch L, Schöffl C, Aberer W, Massone C. Methotrexate Treatment for Pityriasis Rubra Pilaris: A Case Series and Literature Review. Acta Derm Venereol. 2018 Apr 27. 98 (5):501-505. [Medline].

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, Florida Medical Association, Noah Worcester Dermatological Society, Society for Clinical and Experimental Hypnosis, American Contact Dermatitis Society, American Association for Physician Leadership, American Medical Association, American Society of Clinical Hypnosis

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Andrea Leigh Zaenglein, MD Professor of Dermatology and Pediatrics, Department of Dermatology, Hershey Medical Center, Pennsylvania State University College of Medicine

Andrea Leigh Zaenglein, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Received consulting fee from Galderma for consulting; Received consulting fee from Valeant for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Anacor for consulting; Received grant/research funds from Stiefel for investigator; Received grant/research funds from Astellas for investigator; Received grant/research funds from Ranbaxy for other; Received consulting fee from Ranbaxy for consulting.

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.

Pityriasis Rubra Pilaris

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