Faculty Sponsor

Jana Stedman

College

College of Nursing & Health Professions (CONHP)

Department/Program

Physician Assistant

ORCID Identifier(s)

N/A

Presentation Type

Poster Presentation

Symposium Date

Summer 7-22-2026

Abstract

Objective:

The purpose of this literature review was to evaluate the effectiveness and safety of topical retinoid and biological therapies for pediatric patients with Lamellar Ichthyosis. These therapies were compared with standard keratolytic and emollient treatments in improving scaling severity, dryness, pain, pruritus, quality of life, while minimizing infection rates and adverse effects.

Methods:

A narrative literature review was completed using PubMed, EBSCO, Google Scholar, and Research Rabbit Al. Studies in English, within five years, were included if they evaluated pediatric patients with autosomal recessive congenital ichthyosis and had full-text availability. Studies involving adults, non-hereditary ichthyosis, or patients with comorbidities were excluded. Five primary studies, including randomized controlled trials, an interventional study, and case reports, were reviewed background literature.

Results:

Topical isotretinoin therapies, particularly lower concentrations of 0.1% PAT-001 and 0.05% TMB-001, reduced scaling severity without evidence of systemic absorption. Secukinumab improved pruritus by 50%, pain by 66.7%, and quality of life by 60%, but had minimal effect on scaling. Ustekinumb demonstrated variable improvement in disease severity; however, infection concerns, including a 15.4% hospitalization rate for sepsis, had raised safety concerns.

Conclusion:

Topical retinoids reduced localized scaling in pediatrics with Lamellar Ichthyosis. Biological therapies improved pruritus, pain, and quality of life, but minimal improvement in scaling. These therapies should be an adjunct to standard treatment, with management individualized. Larger studies with longer follow-ups are essential to understand their safety and effectiveness in pediatric patients.

Keywords: lamellar ichthyosis; topical isotretinoin; biologics; pediatric dermatology; congenital ichthyosis; scaling severity

Biographical Information about Author(s)

Eileen Uthuppan is a Valparaiso University Physician Assistant Class of 2026 member and rare disease advocate with a special interest in dermatology. She has volunteered with the Foundation for Ichthyosis & Related Skin Types since 2018 and guided by her lived experience, Eileen will explore therapies to improve care for pediatric Lamellar Ichthyosis patients in "Navigating Topical & Biologic Therapies in Pediatric Patients with Lamellar Ichthyosis". 

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