Severe atypical juvenile pityriasis rubra pilaris diagnosed in adulthood with a dramatic improvement with ustekinumab
Abstract Pityriasis rubra pilaris (PRP) is a rare skin disease which manifests as a psoriasiform dermatosis and palmoplantar keratoderma and has significant clinical‐histopathological overlap with psoriasis. Recently, several case reports have demonstrated successful treatment of PRP with anti‐IL7A...
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Main Authors: | , , , , , |
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Format: | Book |
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Wiley,
2024-08-01T00:00:00Z.
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Summary: | Abstract Pityriasis rubra pilaris (PRP) is a rare skin disease which manifests as a psoriasiform dermatosis and palmoplantar keratoderma and has significant clinical‐histopathological overlap with psoriasis. Recently, several case reports have demonstrated successful treatment of PRP with anti‐IL7A and anti‐IL12/anti‐IL23 monoclonal antibodies. We report a case of atypical juvenile PRP definitively diagnosed during adulthood with presence of CARD14 mutation. This case demonstrates a dramatic improvement with ustekinumab and highlights the role of genetic testing in chronic disease of diagnostic uncertainty. |
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Item Description: | 2690-442X 10.1002/ski2.389 |