Juvenile pityriasis rubra pilaris: report of 28 cases in Taiwan
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Published on: December 15, 2011
Chao-Chun Yang1, I-Hsin Shih, Wan-Lung Lin
1Department of Dermatology, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
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.