Recurrent and novel GLTP variants in five patients with nonsyndromic epidermal differentiation disorder: phenotypic and genotypic expansion.

Zhang, Zeqiao; Yang, Chao; Jiang, Xingyuan; Luo, Angela J; Mathes, Erin; Chen, Yongfeng; Choate, Keith A; Lin, Zhimiao et al. · Br J Dermatol · 2026

case_series · Level IV

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Abstract

This study reports four novel and one recurrent biallelic <i>GLTP</i> variants in a new cohort, solidifying its role in nonsyndromic epidermal differentiation disorder (nEDD). The recurrent variant c.58_62del shows a founder effect in the Chinese population. A consistent histopathological feature of <i>GLTP</i>-related nEDD is cytoplasmic vacuolization of keratinocytes associated with autophagic flux impairment, suggesting a potential pathognomonic sign for this condition.