Pityriasis rubra pilaris (overview)L44.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.11.2023

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HistoryThis section has been translated automatically.

Devergie, 1863

DefinitionThis section has been translated automatically.

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.

ClassificationThis section has been translated automatically.

According to Griffith, a distinction is made between 5 types, to which an HIV-associated form is added:

  1. Classic adult type (approx. 50%): 80% recovery after 3 years.
  2. Atypical adult type (approx. 5%): Eminently chronic course.
  3. Classicjuvenile type (approx. 10%): Healing usually after 1 year.
  4. Circumscribed juvenile type (approx. 25%): psoriasis-like aspect; different healing rates (1/3 in 3 years). Often associated with infection.
  5. Atypical juvenile type (approx. 5%): chronic course, limited to palms and soles, occasionally familial (GOF mutation in the CARD14 gene can be detected - see below). CARD14-associated papulosquamous eruption - Craiglow BG et al. 2018 )
  6. HIV-associated form (< 5%).

Occurrence/EpidemiologyThis section has been translated automatically.

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

EtiopathogenesisThis section has been translated automatically.

Unknown.

It is a hyperproliferative keratinization disorder with increased turnover rates.

Mostly sporadic in occurrence.

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

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).

ManifestationThis section has been translated automatically.

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 featuresThis section has been translated automatically.

The disease is characterized by the following main symptoms:

  1. chronic, changeable, also seasonal, course lasting for years
  2. large, red-orange, psoriasiform or ichthyosiform plaques with sharply marked recesses(nappes claires)
  3. extensive palmo-plantar keratoses
  4. follicular keratoses
  5. Histology: diffuse superficial dermatitis, horizontal and vertical parakeratosis with follicular hyperkeratoses

Initial symptoms: Dense, follicular, keratotic, red to red-brownish (orange-red), pinhead-sized, pointed papules with central, conical or cratered keratosis. Most pronounced on the dorsum of the fingers and hands and the extensor sides of the extremities.

Pityriasis rubra pilaris type 1 (classic form): Extensive pityriasiform siliceous, often marginal, sharply demarcated, follicular ("nutmeg grater"), lichenified erythema or plaques on the capillitium, face (especially eyebrows, nasolabial folds), also on the trunk.

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

Pityriasis rubra pilaris type 4 (circumscripte juvenile - psoriasiform - type): Sharply demarcated plaques on the knee and elbow and follicular hyperkeratosis with perifollicular erythema.

Pityriasis rubra pilaris type 5 (atypical juvenile form): This form is characterized by early onset (usually as early as 1 year of age - see below. CARD14-associated papulosquamous eruption - Craiglow BG et al. 2018 )and long disease duration. Sclerodermatous changes of the hands and feet are particularly emphasized.

Complication(s)This section has been translated automatically.

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.

LiteratureThis section has been translated automatically.

  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 immunodeficiency 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. Craiglow BG et al. (2018 ) CARD14 - Associated Papulosquamous Eruption (CAPE): A Spectrum Including Features of Psoriasis and Pityriasis Rubra Pilaris. J Am Acad Dermatol 79: 487-494.
  8. 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
  9. Gemmeke A et al. (2010) Pityriasis rubra pilaris - a retrospective monocentric analysis over 8 years. JDDG 8: 439-445
  10. Haenssle H et al. (2004) Extracorporal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris. Clin Exper Dermatol 29: 244-246
  11. Hashimoto K et al. (1999) Pityriasis rubra pilaris with acantholysis and lichenoid histology. Am J Dermatopathol 21: 491-493
  12. Kaskel P et al (2001) Phototesting and phototherapy in pityriasis rubra pilaris. Br J Dermatol 144: 430
  13. Kaposi M (1895) Lichen ruber acuminatus and lichen ruber planus. Archives of Dermatology and Syphilis (Prague) 31: 1-32
  14. 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.
  15. Martin KL et al. (2014) An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases. Pediatr Dermatol 31:138-145
  16. Mercer JM et al. (2013) Familial pityriasis rubra pilaris: case report and review. J Cutan Med Surg 17:226-232
  17. 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
  18. Nakafusa J et al. (2002) Pityriasis rubra pilaris in association with polyarthritis. Dermatology 205: 298-300
  19. 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
  20. Terheyden P et al. (2001) Erythroderma after corticosteroid withdrawal in papulosquamous dermatosis in children. Diagnosis: pityriasis rubra pilaris, classic juvenile type. Dermatology 52: 1058-1061
  21. Vasher M et al. (2010) Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept. J Drugs
  22. Dermatol 9:844-850
  23. Wetzig T et al. (2003) Juvenile pityriasis rubra pilaris: successfull treatment with cyclosporine. Br J Dermatol 149: 202-203

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Last updated on: 05.11.2023