Pityriasis rubra pilaris (juvenile type) L44.0

Rare, hereditary, chronic inflammatory keratinization disorder with follicular keratoses and extensive palmo-plantar hyperkeratoses. Although the skin changes have a psoriasiform pattern, there is no relationship to psoriasis.

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

1. Classic adult type (approx. 50%): 80% healing after 3 years.
2. Atypical adult type (approx. 5%): Eminently chronic course.
3. Classic juvenile type (approx. 10%): Healing mostly after 1 year.
4. Described juvenile type (approx. 25%): Different healing rates (1/3 in 3 years).
5. Atypical juvenile type (about 5%): Chronic course.
6. HIV-associated form (< 5%).

Pityriasis rubra pilaris type 3: Extensive pityriasiform, often marginal, sharply defined, (not always clinically evident) follicular ("nutmeg rub"), lichenified erythema or plaques on the capillitium, face (especially eyebrows, nasolabial folds, trunk (also anogenital) and extensor extremities including palms of hands and feet. Plaques on the capillitium, face (especially eyebrows, nasolabial folds), trunk (also anogenital) and extensor extremities including palms and soles (palmoplantar keratosis) as well as fingernails and toenails.

Pityriasis rubra pilaris type 4 (circumscripts juvenile - psoriasiform - type): Sharply defined plaques on the knees and elbows and follicular hyperkeratosis with perifollicular erythema. Remark: In this type it is very difficult to differentiate from psoriasis.

Pityriasis rubra pilaris type 5 (atypical juvenile form): This form is characterized by early (possibly even congenital) initial manifestation, long duration of disease and extensive (also erythrodermic) pattern of infestation. Sclerodermic changes of the hands and feet are particularly emphasized. In this type of pityriasis rubra pilaris, heterozygous gain-of-function mutations are found in CARD14, which were not found in other PRP types.

S.u. Pityriasis rubra pilaris (adult type)

1. Dessinioti C et al (2011) Long-term infliximab treatment for refractory type III juvenile pityriasis rubra pilaris. Eur J Dermatol 21:599-600
2. Klein A et al (2010) Pityriasis rubra pilaris: a review of diagnosis and treatment. At J Clin Dermatol 11:157-170
3. Lernia VD et al (2015) Ineffectiveness of tumor necrosis factor-α blockers and ustekinumab in a case of type IV pityriasis rubra pilaris. Indian Dermatol Online J 6:207-209
4. López-Gómez A et al (2015) Dermoscopy of circumscribed juvenile pityriasis rubra pilaris. J Am Acad Dermatol72(1 Suppl):S58-559
5. Martin KL edt al. (2014) An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases. Pediatric Dermatol 31:138-145