Pityriasis rubra pilaris

Pityriasis rubra pilaris

Chris Schach

Author Bio -

 Key Points
*Group of rare skin conditions which may affect any area of the body
*Exact cause is unknown, though some forms may be hereditary
*Consists of clearly defined areas of orange-red scaling on the skin

Pityriasis rubra pilaris (PRP) is a group of rare skin conditions which may affect any area of the body. Generally, the condition consists of clearly defined patches of orange-red scaling on the skin. In most cases, widespread involvement, sometimes of the entire skin surface, occurs. There are several subtypes of the condition.

Classical adult onset PRP is the most common subtype, affects the head, neck and upper torso, and typically resolves spontaneously within 3 years, usually without relapse. Atypical adult onset PRP is rare, and may continue in affected persons for decades. This is usually accompanied by eczema of some type. Classical juvenile onset PRP typically develops between 5 and 10, and follows an infection in most cases. This type of PRP usually resolves within 1 year. Circumscribed juvenile PRP is fairly common, presenting prior to puberty and is typically localized to the palms, soles, elbows and knees. Atypical juvenile onset PRP is rare, and may be inherited. It typically presents at birth or infancy, is extremely resistant to resolution and may be accompanied by ichtyosis. HIV-associated PRP is similar to classical adult onset PRP, but is more resistant to treatment.

While PRP may be associated with certain other disorders, the exact cause of the condition is unknown. Some forms of the condition may also be genetic.

Differential Diagnosis (Other conditions with similar appearance)
Cutaneous T-cell lymphoma
Erythroderma
Erythrokeratodermia Variabilis
Psoriasis, Plaque

Diagnosis
Key Points
*Diagnosis based on the characteristic appearance of the affected area
*Skin biopsy may be performed to confirm diagnosis and rule out other conditions

PRP is typically diagnosed based on the characteristic appearance of the affected area. A skin biopsy will be performed to confirm the diagnosis and to rule out similar appearing conditions.

Treatment
*Treatment is not always necessary, as some forms of the condition are self-resolving
*Genetic forms of the condition are persistent, typically throughout life
*Severe cases may require treatment with acitretin or isotretinoin
OTC Options: emollients, topical steroids

PRP does not always require treatment, as the most common form of the condition is self-resolving. Genetic forms, however, are very persistent, typically throughout the affected person's life. Use of emollients is recommended to relieve any cracking or dryness which may result from the condition. In severe cases, acitretin or isotretinoin may be prescribed to assist in partial resolution.

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