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    J Am Acad Dermatol. 2008 Mar;58(3):505-7.

    Ichthyosis cribriformis: A new entity?

    Larangeira de Almeida H Jr, Happle R, Blume-Peytavi U, de Castro LA.

    Catholic and Federal University of Pelotas, Pelotas, Brazil. hiramalmeidajr@hotmail.com

    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."

    PMID: 18280353 [PubMed - indexed for MEDLINE]

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