Pityriasis Rubra Pilaris
Pityriasis Rubra Pilaris (PRP) is a group of rare, chronic, inflammatory skin disorders characterized by follicular hyperkeratotic papules, waxy yellow palmoplantar keratoderma, and erythematous plaques with islands of sparing. It can affect individuals of all ages, with bimodal peaks in the first and fifth to sixth decades of life, and occurs equally in males and females.
Clinically, PRP typically presents with red-orange scaly patches that begin on the head, neck, and upper trunk. These patches often spread to cover most of the body, including the palms and soles, which become thickened and yellow. The condition can cause significant discomfort, including itching and pain, which may interfere with daily activities. Diagnosis is primarily clinical, supported by biopsy if needed to rule out other conditions. Treatment for PRP is challenging and varies depending on the severity and type of PRP. Options include topical therapies like emollients, corticosteroids, and vitamin D analogs, as well as systemic treatments such as retinoids and immunosuppressants. Biologic agents have also shown promise in treating refractory cases. The prognosis varies: classical adult-onset PRP often resolves within three years, while other types may persist for longer periods.
References
- Wang D, Chong VC, Chong WS, Oon HH. A Review on Pityriasis Rubra Pilaris. Am J Clin Dermatol. 2018;19(3):377-390. doi:10.1007/s40257-017-0338-1
- Napolitano M, Abeni D, Didona B. Biologics for pityriasis rubra pilaris treatment: A review of the literature. J Am Acad Dermatol. 2018;79(2):353-359.e11. doi:10.1016/j.jaad.2018.03.036