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

Autor: Dieter Metze, Heiko Traupe, Kira Süßmuth
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Dermatopathology, Vol 8, Iss 2, Pp 107-123 (2021)
Druh dokumentu: article
ISSN: 2296-3529
DOI: 10.3390/dermatopathology8020017
Popis: 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.
Databáze: Directory of Open Access Journals
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