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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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, 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%).

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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 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).

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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, 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.

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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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Last updated on: 05.11.2023