An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis.

ABSTRACT:

Background

The ichthyoses are rare genetic disorders associated with generalized scaling, erythema, and epidermal barrier impairment. Pathogenesis-based therapy is largely lacking because the underlying molecular basis is poorly understood.

Objective

We sought to characterize molecularly cutaneous inflammation and its correlation with clinical and barrier characteristics.

Methods

We analyzed biopsy specimens from 21 genotyped patients with ichthyosis (congenital ichthyosiform erythroderma, n = 6; lamellar ichthyosis, n = 7; epidermolytic ichthyosis, n = 5; and Netherton syndrome, n = 3) using immunohistochemistry and RT-PCR and compared them with specimens from healthy control subjects, patients with atopic dermatitis (AD), and patients with psoriasis. Clinical measur

SUBMITTER: Paller AS 

PROVIDER: S-EPMC8033419 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature