Ichthyosis cribriformis: A new entity?

Larangeira de Almeida, Hiram; Happle, Rudolf; Blume-Peytavi, Ulrike; de Castro, Luis Antônio Suita · J Am Acad Dermatol · 2008

case_report · Level V

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Abstract

A 22-year-old woman had an unusual congenital skin disorder in the form of ichthyosis characterized by innumerable keratotic plugs, resulting in a peculiar sievelike appearance. On histopathologic examination the epidermis showed a pronounced orthohyperkeratosis. The keratotic plugs seemed to emerge from the infundibular region of hair follicles. Immunohistochemical characterization revealed a normal keratin and filaggrin expression. Oral treatment with isotretinoin resulted in a reduction of the hyperkeratosis. Because we could not find any previously reported similar case, we propose the new name "ichthyosis cribriformis," which means "sievelike ichthyosis."

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