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, 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%).

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 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).

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, 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.

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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