Pityriasis rubra pilaris (overview) L44.0

Devergie, 1863

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.

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%).

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

Unknown.

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).

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.

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.

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.

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.