Ichthyosis linearis circumflexa Comel with Trichorrhexis invaginata (Netherton's Syndrom) an ultrastructural study of the skin changes

E Frenk, B Mevorah - Archiv für dermatologische Forschung, 1972 - Springer
E Frenk, B Mevorah
Archiv für dermatologische Forschung, 1972Springer
An electron microscopical study of skin from 2 patients with ichthyosis linearis circumflexa
and trichorrhexis invaginata revealed a severe and localized disturbance of keratinization
occuring at the keratotic border of figurate skin lesions. It is characterized by a marked
decrease of the desmosome-tonofilament system, lack of membrane coating granules and
keratohyalin, and the appearence of roundoval, electron dense bodies in the upper spinous
layers. The result is a completely disorganized “stratum corneum”. We consider this unique …
Summary
An electron microscopical study of skin from 2 patients with ichthyosis linearis circumflexa and trichorrhexis invaginata revealed a severe and localized disturbance of keratinization occuring at the keratotic border of figurate skin lesions. It is characterized by a marked decrease of the desmosome-tonofilament system, lack of membrane coating granules and keratohyalin, and the appearence of roundoval, electron dense bodies in the upper spinous layers. The result is a completely disorganized “stratum corneum”. We consider this unique keratinization disorder to be specific for this disease.
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