Pityriasis Rubra Pilaris: A Report of Two Cases and Literature Review

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Abstract

Pityriasis rubra pilaris (PRP) is an idiopathic inflammatory hyperproliferative chronic dermatosis characterized by: perifollicular coalescing papules with central keratotic acuminate plugs gradually submerged in sheets of erythema; perifollicular erythema with islands of unaffected skin; palmoplantar keratoderma; diffuse desquamation which typically spreads from the head down to the feet. The cause of the condition is unknown, but possible etiological factors include: vitamin A deficiency, trauma, infections, autoimmune mechanisms, and malignancies. Taking into account different age of onset, clinical course, morphology and prognosis, there are six different types of the disease: two in adults (classical and atypical); three in children (classical, circumscribed and atypical); one in individuals infected with human immunodeficiency virus.

This paper presents two male patients with clinical symptoms of classical PRP, 53 and 69 years of age at the onset of the disease, with rapid generalized involvement, typical erythematous perifollicular papules, islands of unaffected skin, palmoplantar hyperkeratosis with a waxy appearance and nail changes. The diagnosis was based on clinical findings and histopathologic analysis. Apart from topical therapy with emollients, corticosteroids and keratolytics, they received systemic retinoids and corticosteroids, which resulted in improvement of skin lesions.

It is extremely important to consider the possible triggering factors, establish the diagnosis as soon as possible and begin proper treatment.

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