Juvenile pityriasis rubra pilaris: Report of 28 cases in Taiwan - 24/04/13
Abstract |
Background |
Pityriasis rubra pilaris (PRP) is a papulosquamous dermatosis uncommon in juveniles. Large-scale studies are limited, especially from Asian countries.
Objective |
We sought to analyze the clinical manifestations of juvenile PRP in Taiwanese patients and compare them with reported series in the literature.
Methods |
The diagnosis of juvenile PRP was made based on clinical–histopathologic correlation. The therapeutic response and disease course were followed up by re-examination of the patients or by telephone.
Results |
A total of 47 patients were identified, with histopathologic confirmation of the clinical diagnosis of juvenile PRP in 28 cases. A preponderance of Griffiths’ type IV PRP (85.7%) rather than type III PRP (14.3%) was found. Palmoplantar hyperkeratosis appeared to be a cardinal feature. In patients with type IV PRP, skin lesions in areas other than the elbows/knees and palms/soles were common. Treatment with systemic acitretin in 6 patients failed to effect a dose- or time-dependent improvement. In contrast with other studies, two thirds of our patients with type III and IV juvenile PRP had a protracted course lasting more than 3 years.
Limitations |
This study was a retrospective review. Patient compliance with treatment was frequently poor.
Conclusions |
Type IV juvenile PRP predominated but our cases showed a wider distribution of skin lesions than is typically described. When children present with an acute onset of diffuse palmoplantar hyperkeratosis, a diagnosis of juvenile PRP should be considered. Because of the divergent clinical manifestations of juvenile PRP in different populations, there is a need to modify and re-evaluate classification systems based on regional differences.
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Funding sources: None. |
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Conflicts of interest: None declared. |
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Presented as a poster at the 66th Annual Meeting of the American Academy of Dermatology, San Antonio, Texas, February 1-5, 2008. |
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Reprints not available from the authors. |
Vol 59 - N° 6
P. 943-948 - décembre 2008 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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