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

Dermatopathology

Heiko Traupe¹  Dieter Metze¹  Kira Süßmuth¹ 

[1] Klinik für Hautkrankheiten, Universitätsklinik Münster, 48149 Münster, Germany;

关键词: ichthyosis;    hereditary keratinization disorders;    dermatopathology;    pattern analysis;    immunohistochemistry;   

DOI  :  10.3390/dermatopathology8020017

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown