Pityriasis Rubra Pilaris: A Report of Two Cases and Literature Review

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Pityriasis rubra pilaris (PRP) is an idiopathic inflammatory hyperproliferative chronic dermatosis characterized by: perifollicular coalescing papules with central keratotic acuminate plugs gradually submerged in sheets of erythema; perifollicular erythema with islands of unaffected skin; palmoplantar keratoderma; diffuse desquamation which typically spreads from the head down to the feet. The cause of the condition is unknown, but possible etiological factors include: vitamin A deficiency, trauma, infections, autoimmune mechanisms, and malignancies. Taking into account different age of onset, clinical course, morphology and prognosis, there are six different types of the disease: two in adults (classical and atypical); three in children (classical, circumscribed and atypical); one in individuals infected with human immunodeficiency virus.

This paper presents two male patients with clinical symptoms of classical PRP, 53 and 69 years of age at the onset of the disease, with rapid generalized involvement, typical erythematous perifollicular papules, islands of unaffected skin, palmoplantar hyperkeratosis with a waxy appearance and nail changes. The diagnosis was based on clinical findings and histopathologic analysis. Apart from topical therapy with emollients, corticosteroids and keratolytics, they received systemic retinoids and corticosteroids, which resulted in improvement of skin lesions.

It is extremely important to consider the possible triggering factors, establish the diagnosis as soon as possible and begin proper treatment.

1. Judge MR, McLean WHI, Munro CS. Disorders of keratinization. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s textbook of dermatology. 8th ed. Oxford: Blackwell Publishing Ltd; 2010. p. 19.1-19.122.

2. Katsambas A, Dessinioti C. Pityriasis rubra pilaris. © 2016 UpToDate, Inc [updated 2016 Jan 4; cited 2016 Feb 5]. Available from: http://www.uptodate.com/contents/pityriasis-rubra-pilaris

3. Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. Int J Dermatol 2006;45(4):438-46.

4. Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol 2008;74(4):311-6.

5. Tarral C. General psoriasis. Desquamation from the parts covered with hair. In: Rayer PA. Theoretical and practical treatise on the diseases of the skin. 2nd ed. London: Baillier; 1835. p. 648-9.

6. Devergie MGA. Pityriasis pilaris, maladie de peau non decrite par les dermatologistes. Gazette Hebdomadaire de medecine et chirurgie 1856;3:197-201.

7. Richaud A, Féré C. Etude sur le pityriasis pilaris [dissertation]. Paris: A Parent; 1877.

8. Besnier E. Observation pour servir a l’histoire clinique du Pityriasis rubra pilaire. Ann Dermatol Syphiligr (Paris) 1889;10(ser 2):454-64.

9. De Beurmann, Bith, Heuyer. Pityriasis rubra pilaire familial. Ann Dermatol Syphiligr (Paris) 1910;1(ser 5):606-19. (Note: No initials given in original text).

10. Shenefelth PD. Pityriasis rubra pilaris: clinical presentation. Medscape.com [homepage on the Internet]. © 1994-2016 by WebMD LLC [updated 2016 Feb 25; cited 2016 Feb 29]. Available from: http://reference.medscape.com/article/1107742-clinical

11. Vijayalakshami AM, Mallika A. Pityriasis rubra pilaris. Indian Pediatr 2003;40(5):432-3.

12. Vasher M, Smithberger E, Lien MH, Fenske NA. Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept. J Drugs Dermatol 2010;9(7):844-50.

13. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980;5(1):105-12.

14. Larregue M, Champion R, Bressieux JM, Laidet B, Lorette G. Acute pityriasis rubra pilaris in the child. Apropos of 4 cases. Ann Dermatol Venereol 1983;110(3):221-8.

15. Ferrandiz-Pullido C, Bartralot R, Bassas P, Bodet D, Repiso T, Aparicio G, et al. Acute postinfectious pityriasis rubra pilaris: a superantigen-mediated dermatosis. Actas Dermatosifiliogr 2009;100(8):706-9.

16. Piamphongsant T, Akaraphant R. Pityriasis rubra pilaris: a new proposed classification. Clin Exp Dermatol 1994;19(2):134-8.

17. Miralles ES, Nufiez M, De Las Heras ME, Pérez B, Moreno R, Ledo A. Pityriasis rubra pilaris and human immunodeficiency virus. Br J Dermatol 1995;133(6):990-3.

18. Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review of diagnosis and treatment. Am J Clin Dermatol 2010;11(3):157-70.

19. Betto P, Vassilopoulou A, Colombari R, Veller-Fornasa C. Acute juvenile pityriasis rubra pilaris: a case report after mononucleosis infection. G Ital Dermatol Venereol 2008;143(4):271-3.

20. Chowdhary M, Davila U, Cohen DJ. Ustekinumab as an alternative treatment option for chronic pityriasis rubra pilaris. Case Rep Dermatol 2015;7(1):46-50.

21. Kawara S, Miyake M, Oiso N, Kawada A. Pityriasis rubra pilaris with preceding cytomegalovirus infection. Dermatology 2009;219(4):350-2.

22. Kivi R, Kinman T. Pityriasis rubra pilaris. Healthline.com © 2005 - 2016 Healthline Media. [cited 2016 Feb 29]. Available from: http://www.healthline.com/health/pityriasis-rubrapilaris#Overview1

23. Solanki LS, Srivastava N, Singh S. Superantigens: a brief review with special emphasis on dermatologic diseases. Dermatol Online J 2008;14(2):3.

24. Ertam I, Sezgin AO, Kasandi A, Dereli T, Unal I. A case of juvenile pityriasis rubra pilaris: could varicella be an aetiological agent? Clin Exp Dermatol 2009;34(8):e1012-3.

25. Naciri Bennani B, Cheikh Rouhou H, Waton J, Cuny JE, Bassegoda G, Trechot P, et al. Pityriasis rubra pilaris after vaccination. Ann Dermatol Venereol 2011;138(11):753-6.

26. Weismann K. A 12-year-old girl with a scaly papular rash. Forum for Nord Derm Ven 2010;15(2):54-5.

27. De D, Dogra S, Narang T, Radotra BD, Kanwar AJ. Pityriasis rubra pilaris in a HIV-positive patient (Type 6 PRP). Skinmed 2008;7(1):47-50.

28. Fuchs-Telem D, Sarig O, van Steensel MA, Isakov O, Israeli S, Nousbeck J, et al. Familial pityriasis rubra pilaris is caused by mutations in CARD14. Am J Hum Genet 2012;91(1):163-70.

29. Jordan CT, Cao L, Roberson ED, Pierson KC, Yang CF, Joyce CE, et al. PSORS2 is due to mutations in CARD14. Am J Hum Genet 2012;90(5):784–95.

30. Martinez Calixto LE, Suresh L, Matsumura E, Aguiere A, Radfar L. Oral pityriasis rubra pilaris. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101(5):604-7.

31. Martin Callizo C, Molinero Caturla J, Sanchez Sanchez J, Penin Mosquera RM. Scarring alopecia in classic adult type I pityriasis rubra pilaris. Actas Dermosifiliogr 2014;105(10):955-7.

32. Abbott RA, Griffiths WA. Pityriasis rubra pilaris type I spontaneously resolving after 20 years. Clin Exp Dermatol 2009;34(3):378-9.

33. Arnold AW, Buechner SA. Circumscribed juvenile pityriasis rubra pilaris. J Eur Acad Dermatol Venereol 2004;8(6):705-7.

34. Yang CC, Shin IH, Lin WL, Yu YS, Chiu HC, Huang PH, et al. Juvenile pityriasis rubra pilaris: report of 28 cases in Taiwan. J Am Acad Dermatol 2008;59(6):943-8.

35. Mobini N, Toussaint S, Kamino H. Noninfectious erythematous, papular, and squamous disease. In: Elder DE, Elenitsas R, Johnson B, Murphy G, editors. Lever’s histopathology of the skin. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 179-214.

36. Jackson S, Nesbitt LT. Differential diagnosis in dermatology for the dermatologists. 2nd ed. Berlin, Heidelberg: Springer; 2012.

37. Koley S, Choudhary S, Salodkar A, Sarkar J, Choudhary M. Circumscribed juvenile type pityriasis rubra pilaris (type 4): are we missing them? Journal of Pakistan Association of Dermatologists 2010;20:98-102.

38. Kubanov A, Gallyamova Y. Diagnosis and treatment of pityriasis rubra pilaris. Serbian Journal of Dermatology and Venereology 2014;6(4):167-73.

39. Kitzmann AS, Goins KM, Syed NA, Wagoner MD. Bilateral herpes simplex keratitis with unilateral secondary bacterial keratitis and corneal perforation in a patient with pityriasis rubra pilaris. Cornea 2008;27(10):1212-4.

40. Word AP, Patel M, Menter AM. A novel case of recalcitrant juvenile pityriasis rubra pilaris with response to bexarotene. J Clin Exp Dermatol Res 2011;2(7):131.

41. Mattox AR Chappell JA, Hurley MY. New-onset vitiligo during long-term, stable infliximab treatment of pityriasis rubra pilaris. J Drug Dermatol 2013;12(2):217-9.

42. Ivanova K, Itin P, Haeusermann P. Pityriasis rubra pilaris: treatment with biologics - a new promising therapy? Dermatology 2012;224(2):120-5.

43. Di Stefani A, Galluzzo M, Talamonti M, Chirocozzi A, Contanzo A, Chimenti S. Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris. J Dermatol Case Rep 2013;7(1):5-9.

44. Lombardi V, Rossi MM, Minvielle AI. Pityriasis rubra pilaris. Arch Argent Dermatol 2013:63(6):254-62.

45. Gajinov ZT, Matić MB, Đuran VD, Vučković N, Prcić ST, Vujanović LM. Drug-related pityriasis rubra pilaris with acantholysis. Vojnosanit Pregl 2013;70(9):871-3.

46. Muller H, Gattringer C, Zelger B, HopflR, Eisendle K. Infliximab monotherapy as first-line treatment for adultonset pityriasis rubra pilaris: case report and review of the literature on biologic therapy. J Am Acad Dermatol 2008;59(5 Suppl):S65-70.

47. Amann PM, Susic M, Gluder F, Berger H, Krapf W, Loffler H. Alitretinoin (9-cis retinoic acid) is effective against pityriasis rubra pilaris: a retrospective study. Acta Derm Venereol 2015;95(3):329-31.

48. Vergilis–Kalner IJ, Mann DJ, Wassermann J, Petronić-Rosić V, Tsoukas MM. Pityriasis rubra pilaris sensitive to narrow band-ultraviolet B light therapy. J Drugs Dermatol 2009;8(3):270-3.

49. Bexaroten. Flexicon.doccheck.com [homepage on the Internet]. ©2016 DocCheck Medical Services GmbH. [cited 2016 Feb 28]. Available from: http://flexicon.doccheck.com/de/Bexaroten

50. Petrof G, Almaani N, Archer CB, Griffiths WA, Smith CH. A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF-antagonists. J Eur Acad Dermatol Venereol 2013;27(1):e131-5.

51. Eastham AB, Femia AN, Qureshi A, Vleugels RA. Treatment options for pityriasis rubra pilaris including biologic agents: a retrospective analysis from an academic medical center. JAMA Dermatol 2014;150(1):92-4.

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