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Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that occurs with phenotypic variability in adults of all ages as well as in children. Data on the treatment of PRP is limited. Here, we report a 5‐year‐old girl with widespread skin involvement and prominent palmoplantar hyperkeratosis who was initially treated for psoriasis. After reevaluation, a diagnosis of PRP was made, and the patient had an excellent therapeutic response to ustekinumab and acitretin.

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