Debilitating Contractures of Hands in an Atypical Presentation of Juvenile Pityriasis Rubra Pilaris: A Rare Case Report

Abstract

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, keratodermas have been categorised as hereditary and acquired forms. Case Presentation: A 12-year-old male patient presented to our dermatology outpatient department with diffuse thickening and yellowish discoloration of skin over palms and soles since early childhood. He gradually developed progressive thickening and contractures of the fingers of both hands with functional impairment. No such history could be elicited in the family members. Dermatological examination showed bilaterally symmetrical thick hyperkeratotic fissured scaly plaques involving surface of entire palm with sharp demarcation at volar aspect of wrist. Similar plaques with yellowish hue were present over the soles with superficial fissures. The histopathological examination of skin biopsy specimen from the palm showed focal parakeratosis alternating with orthokeratosis in both horizontal and vertical directions (checkerboard pattern) with broadened rete ridges. The findings were consistent with pityriasis rubra pilaris (PRP). The patient improved with systemic and topical retinoids. Conclusions: This report focuses on the recognition of unusual presentations of PRP and emphasizes the importance of early diagnosis and timely treatment, which can avert the dreaded morbid and disabling complication of contractures.