Juvenile pityriasis rubra pilaris - Skin
Juvenile pityriasis rubra pilaris (PRP) is a chronic disorder of cornification with onset in early childhood. PRP is usually acquired, but there are heritable forms. It can present in 3 forms: type III (acute), type IV (localized), and type V (atypical).
In type III, there is fairly rapid onset of extensive, confluent, scaling plaques in association with thick keratoderma of the hands and feet. It often begins in the scalp and rapidly involves the trunk and extremities. Type IV is usually limited to the knees and elbows. Type V is present from birth.
L44.0 – Pityriasis rubra pilaris
238620000 – Classical juvenile pityriasis rubra pilaris
- Psoriasis has a more adherent scale, and larger lesions do not have areas of normal skin within them.
- Keratosis pilaris is usually limited to the extensor extremities.
- Erythrokeratodermia variabilis
- Drug eruption
- Atopic dermatitis
- Contact dermatitis
- Sezary syndrome (rare in children; see cutaneous T-cell lymphoma)
- Type V can be confused with lamellar ichthyosis.