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Journal of the American Academy of Dermatology
Volume 58, n° 3
pages 505-507 (mars 2008)
Doi : 10.1016/j.jaad.2007.03.034
Case Reports

Ichthyosis cribriformis: A new entity?

Hiram Larangeira de Almeida, MD a, , Rudolf Happle, MD b, Ulrike Blume-Peytavi, MD c, Luis Antônio Suita de Castro, MSc d
a Catholic and Federal University of Pelotas, Pelotas, Brazil 
b Philipp University of Marburg, Marburg, Germany 
c Department of Dermatology and Allergy Charité-Universitätsmedizin Berlin, Berlin, Germany 
d Laboratory for Electron Microscopy, EMBRAPA-CPA-CT, Pelotas, Brazil 

Reprint requests: Hiram Larangeira de Almeida Jr, MD, Mestrado em Saúde e Comportamento–UCPEL, Rua Barroso 1202–107, 96010-280 Pelotas RS Brazil.

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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 Funding sources: None.
 Conflicts of interest: None declared.

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