Muciño MEY, Díaz MVL, Moreno LLM, Arboleda HVA

Language: Spanish
References: 21
Page: 267-278
PDF size: 553.07 Kb.

ABSTRACT

Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous dermatosis characterized by hyperkeratotic follicular papules, orange-red or salmon-colored plaques with characteristic “islands” of spared skin, and palmoplantar keratoderma. Its clinical heterogeneity and symptomatic overlap with other papulosquamous diseases often complicate diagnosis and management. This review integrates recent evidence regarding its genetic basis—including CARD14 mutations and keratinization-related alterations, particularly in familial and juvenile forms—and its immunological mechanisms, such as the activation of the IL-23/IL-17, TNF-α, and IL-1 pathways. Additionally, histopathological findings and current therapeutic strategies (retinoids, immunomodulators, and targeted biological therapies for refractory cases) are discussed. Ultimately, PRP should be viewed as a heterogeneous spectrum where a subset of cases may be classified as an inflammatory genodermatosis, a conceptualization that carries significant implications for clinical stratification and personalized therapeutic decision-making.

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