Ichthyoses—A Clinical and Pathological Spectrum from Heterogeneous Cornification Disorders to Inflammation

Author:

Metze Dieter,Traupe Heiko,Süßmuth Kira

Abstract

Ichthyoses are inborn keratinization disorders affecting the skin only (non-syndromic) or are associated with diseases of internal organs (syndromic). In newborns, they can be life-threatening. The identification of the gene defects resulted in reclassification and a better understanding of the pathophysiology. Histopathologic patterns include orthohyperkeratosis with a reduced or well-developed stratum granulosum, hyperkeratosis with ortho- and parakeratosis with preserved or prominent stratum granulosum, and epidermolytic ichthyosis. Another pattern features “perinuclear vacuoles and binucleated keratinocytes”, which is associated with keratin mutations. Some ichthyoses are histologically defined by psoriasis-like features, and distinct subtypes show follicular hyperkeratosis. In addition to histological and immunohistochemical methods, these patterns allow a better histopathologic diagnosis.

Publisher

MDPI AG

Subject

General Engineering

Cited by 11 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Advances in the treatment of autosomal recessive congenital ichthyosis, a look towards the repositioning of drugs;Frontiers in Pharmacology;2023-11-09

2. Patients with keratinization disorders due to ABCA12 variants showing pityriasis rubra pilaris phenotypes;The Journal of Dermatology;2023-09-27

3. Advanced Anterior Eye Segment Imaging for Ichthyosis;Journal of Clinical Medicine;2023-09-16

4. Ichthyosis: presentation and management;Current Opinion in Pediatrics;2023-06-22

5. Ichthyosis;Nature Reviews Disease Primers;2023-01-19

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