Recalibrated Scales: The Use of Low-dose Isotretinoin in a Case of Epidermolytic Ichthyosis-NPS1 in a Filipino Child

  • Erickah Mary Therese R. Dy Department of Dermatology, Philippine General Hospital, University of the Philippines Manila Manila
  • Shahara Abalos-Babaran Department of Dermatology, Philippine General Hospital, University of the Philippines Manila
  • Monette R. Faner Department of Dermatology, Philippine General Hospital, University of the Philippines Manila
  • Carmela Augusta F. Dayrit-Castro Department of Dermatology, Philippine General Hospital, University of the Philippines Manila
Keywords: epidermolytic hyperkeratosis, isotretinoin

Abstract

Epidermolytic Ichthyosis (EI) is a rare non-syndromic keratinopathic ichthyosis without definitive treatment. This is a case of EI in a 5-year-old Filipino female who presented with hyperkeratotic scales sparing the palms and soles. Histopathology revealed epidermolytic hyperkeratosis. A trial of treatment with isotretinoin 0.3 mg/kg/day, together with keratolytic agents, urea lotion and lactic acid lotion, resulted in a marked decrease in the thickness of the scales and odor. Interestingly, rebound effects were noted at 0.6 mg/kg/day. Taking into account that EI presents with more skin fragility compared to non-EHK ichthyosis, the authors surmise that there may be a smaller treatment window for patients with EI, which is notably lower than recommended for ichthyosis in general.

Published
2021-08-27