Debilitating Contractures of Hands in an Atypical Presentation of Juvenile Pityriasis Rubra Pilaris: A Rare Case Report
نویسندگان
چکیده
Introduction: Palmoplantar keratodermas (PPK) constitute a clinically and genetically complex group of cornification disorders characterized by defective epidermal differentiation leading to marked palmoplantar hyperkeratosis. Classically, have been categorised as hereditary acquired forms. Case Presentation: A 12-year-old male patient presented our dermatology outpatient department with diffuse thickening yellowish discoloration skin over palms soles since early childhood. He gradually developed progressive contractures the fingers both hands functional impairment. No such history could be elicited in family members. Dermatological examination showed bilaterally symmetrical thick hyperkeratotic fissured scaly plaques involving surface entire palm sharp demarcation at volar aspect wrist. Similar hue were present superficial fissures. The histopathological biopsy specimen from focal parakeratosis alternating orthokeratosis horizontal vertical directions (checkerboard pattern) broadened rete ridges. findings consistent pityriasis rubra pilaris (PRP). improved systemic topical retinoids. Conclusions: This report focuses on recognition unusual presentations PRP emphasizes importance diagnosis timely treatment, which can avert dreaded morbid disabling complication contractures.
منابع مشابه
Case Report Circumscribed juvenile pityriasis rubra pilaris
Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory dermatosis of unclear aetiology. Its most remarkable clinical findings are palmoplantar hyperkeratosis, orange-red scaling plaques and hyperkeratotic follicular papules. Its extension may vary from localised subtypes to generalised skin involvement with well-demarcated areas of spared skin ("islands of sparing") to erythr...
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ژورنال
عنوان ژورنال: Journal of skin and stem cell
سال: 2022
ISSN: ['2423-7086']
DOI: https://doi.org/10.5812/jssc-130311