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."
MeSH terms
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Administration, Oral
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Adult
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Dermatologic Agents / administration & dosage
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Dermatologic Agents / therapeutic use
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Dose-Response Relationship, Drug
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Drug Administration Schedule
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Female
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Filaggrin Proteins
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Humans
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Ichthyosis / classification*
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Ichthyosis / complications
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Ichthyosis / pathology*
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Isotretinoin / administration & dosage
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Isotretinoin / therapeutic use
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Keratoderma, Palmoplantar, Epidermolytic / drug therapy
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Keratoderma, Palmoplantar, Epidermolytic / etiology*
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Keratoderma, Palmoplantar, Epidermolytic / pathology*
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Microscopy, Electron
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Terminology as Topic
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Treatment Outcome
Substances
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Dermatologic Agents
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FLG protein, human
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Filaggrin Proteins
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Isotretinoin