Pityriasis rubra pilaris (overview) L44.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.07.2022

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Devergie, 1863

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Rare, predominantly acquired, chronic inflammatory cornification disorder with follicular keratoses and extensive palmo-plantar hyperkeratoses associated with skin disease of unknown etiology. Although the skin lesions show a psoriasiform pattern, there is no etiopathogenic relationship to psoriasis.

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According to Griffith, 5 types are distinguished, to which an HIV-associated form is added:

  1. Classic adult type (about 50%): 80% cure after 3 years.
  2. Atypical adult type (about 5%): Eminently chronic course.
  3. Classic juvenile type (approx. 10%): Healing usually after 1 year.
  4. Circumscribed juvenile type (approx. 25%): psoriasis-like aspect; variable healing rates (1/3 in 3 years). Not infrequently infection-associated.
  5. Atypical juvenile type (about 5%): chronic course, limited to palms and soles, occasionally familial (association with CARD14).
  6. HIV-associated form (< 5%).

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Incidence: 0.2/100,000/year; prevalence 2/100,000 population.

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It is a hyperproliferative keratinization disorder with increased turnover rates.

Mostly sporadic in onset.

Type 5 (hereditary, atypical juvenile type) is inherited autosomal recessively. Autosomal dominant inheritance has been demonstrated in individual cases. In these, a gain-of-function (GOF)mutation in the CARD14 gene could be detected (Lwin et al. 2018).

Hormonal disorders are discussed.

Retinol-binding protein deficiency and disturbances in vitamin A metabolism have been described.

Viral inducers: similar to psoriasis, the occurrence of pityrisis rubra pilaris is also observed after infections(EBV, CMV, VZV, hepatitis C (Cecchi R et al. 1994) and HIV).

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Basically possible in any age group (see also classification according to juvenile and adult types); in the adult type, clusters are found in the 5th-6th decade of life.

Clinical features
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The disease is characterized by the following main symptoms:

  1. chronic, changeable, also seasonal, yearly course
  2. large, red-orange, psoriasiform or ichthyosiform plaques with sharply marked recesses(nappes claires)
  3. laminar palmo-plantar keratoses
  4. follicular keratoses
  5. Histology: horizontal and vertical parakeratosis with follicular hyperkeratosis

Initial symptoms: Densely packed, follicular, keratotic, red to reddish-brownish (orange-red), pinhead-sized, pointed papules with central, conical or crater-shaped dropping keratosis. Pronounced especially on the back of the fingers and hands as well as the extensor sides of the extremities.

Pityriasis rubra pilaris type 2 and 3: Types 2 and 3 are morphologically not different from the classical adult type.

Pityriasis rubra pilaris type 4 (circumscript juvenile - psoriasiform - type): Sharply defined plaques on knees and elbows and follicular hyperkeratosis with perifollicular erythema.

Pityriasis rubra pilaris type 5 (atypical juvenile form): This form is characterized by early onset and long duration of the disease. Sclerodermatic changes of the hands and feet are particularly prominent.

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Especially in adults the disease can take an acute course. Complicated is the development of erythroderma.

Development of an ectropion, especially in erythrodermatic pityriasis rubra pilaris.

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  1. Albert MR (1999) Pityriasis rubra pilaris. Int J Dermatol 38: 1-11
  2. Auffret N et al (1992) Pityriasis rubra pilaris in a patient with human immunodeficieny virus infection. J Am Acad Dermatol 27: 260-261.
  3. Behr FD et al (2002) Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up. Pediatr Dermatol 19: 46-51
  4. Besnier E (1889) Observations pour servis a l'histoire clinique de pityriasis rubra pilaris. Annales de Dermatologie et de Syphilographie (Paris) 11: 253-287, 398-427, 485-544.
  5. Cecchi R et al (1994) Pityriasis rubra pilaris, lichen planus, alopecia universalis and vitiligo in a patient with chronic viral hepatitis C. Dermatology 188:239-240.

  6. Coras B et al. (2005) Fumaric acid esters therapy: a new reatment modality in pityriasis rubra pilaris? Br J Dermatol 152: 386-403
  7. Devergie MGA (1856) Pityriasis pilaris, maladie de la peau non décrite par les dermatologistes. Gazette hebdomadaire de médecine et de chirurgie (Paris) 3: 197-201.
  8. Gemmeke A et al (2010) Pityriasis rubra pilaris - a retrospective monocentric analysis over 8 years. JDDG 8: 439-445
  9. Haenssle H et al (2004) Extracorporal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris. Clin Exper Dermatol 29: 244-246
  10. Hashimoto K et al (1999) Pityriasis rubra pilaris with acantholysis and lichenoid histology. Am J Dermatopathol 21: 491-493.
  11. Kaskel P et al (2001) Phototesting and phototherapy in pityriasis rubra pilaris. Br J Dermatol 144: 430
  12. Kaposi M (1895) Lichen ruber acuminatus and lichen ruber planus. Archives of Dermatology and Syphilis (Prague) 31: 1-32.
  13. Lewin SM et al (2018) Beneficial effect of ustekinumab in familial pityriasis rubra pilaris with a newmissense mutation in CARD14. Br J Dermatol 178:969-972.
  14. Martin KL et al (2014) An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases. Pediatr Dermatol 31:138-145.
  15. Mercer JM et al (2013) Familial pityriasis rubra pilaris: case report and review. J Cutan Med Surg 17:226-232
  16. Mohrenschlager M et al (2002) Further clinical evidence for involvement of bacterial superantigens in juvenile pityriasis rubra pilaris (PRP): report of two new cases. Pediatr Dermatol 19: 569
  17. Nakafusa J et al (2002) Pityriasis rubra pilaris in association with polyarthritis. Dermatology 205: 298-300
  18. Tarral C (1835) General psoriasis. Desquamation from the part covered with hair. In: Rayer P (editor) A theoretical and practical treatise on the diseases of the skin. Bailliere (London): 648-649
  19. Terheyden P et al (2001) Erythroderma after corticosteroid withdrawal in papulosquamous dermatosis in children. Diagnosis: pityriasis rubra pilaris, classic juvenile type. Dermatologist 52: 1058-1061
  20. Vasher M et al (2010) Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept. J Drugs
  21. Dermatol 9:844-850
  22. Wetzig T et al (2003) Juvenile pityriasis rubra pilaris: successful treatment with cyclosporine. Br J Dermatol 149: 202-203.


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Last updated on: 07.07.2022