Pityriasis Rubra Pilaris

Pityriasis rubra pilaris
  • It is a chronic papulosquamous disorder of unknown etiology characterized by : 
  • Scaling (Pityriasis means scaling Skin disorder)
  • Erythmatous plaque (Rubra)
  • Follicular papules (Pilaris)
Hence the name pityriasis rubra pilaris.
  1. Two peaks of age are seen : 
  2. 5-10 years in juvenile type
  3. 40 - 60 years in adult type
Clinical presentation
  • Characteristic lesions are erythmatous (orange to pink), follicular, scaly plaques. A characteristic feature of plaques is the presence of distinct islands of normal skin. Sites of predilection:
  • Trunk → Lesions on the trunk evolve in craniocaudal (Cephalo caudal) direction. Typically, follicular lesions are seen on the dorsum of hands on knuckles → Nutmeg papules. Associated features
  • Diffuse erythema and scaling of face.
  • Orangish thickening of palms and soles (Keratodermic sandals).
  • Nails  → Distal yellow brown discoloration and nail plate thickening.
  • Complications: Erythroderma
Treatment:
  • Localized lesions → Topical corticosteroids + Keratolytics (Salicylic acid, urea)
  • Erythroderma -4 Vitamin A, Acitretin (Retinoids), oral methotrexate.
Exam Question
  • Isolated patches of normal skin are found in Pityriasis Rubra Pilaris.
  • Cephalocaudal distribution is seen in Pityriasis Rubra Pilaris.
  • Exfoliative dermatitis is seen in Pityriasis Rubra Pilaris.
  • Keratodermic sandles is a feature of Pityriasis Rubra Pilaris.
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