A Rare Case of Incontinentia Pigmenti with Severe Extracutaneous Manifestations
نویسندگان
چکیده
Incontinentia pigmenti (IP) is a complex genodermatosis inherited in an X-linked dominant pattern, associating multistadial cutaneous manifestations with an oculo-dento-cerebral syndrome, which affects only female newborns, as the disorder is lethal in males since intrauterine period. We report on a case of incontinentia pigmenti with an atypical debut consisting of tonic-clonic seizures which had started during the first days of life, associated with severe ocular and neurologic manifestations. The presence and severity of extra-cutaneous features most often command the evolution and the prognosis of the disease.
منابع مشابه
Incontinentia Pigmenti
Incontinentia pigmenti (IP) is an uncommon X-linked dominant genodermatosis characterized by four cutaneous stages and frequent association with dental (90%), central nervous system (33%) and ocular (35%) anomalies. The exact pathogenesis of this disorder remains unknown.Herein, we report a newborn girl with inflammatory vesiculobullous and warty skin lesions and a positive family history of IP...
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Among nevoid causes of hypopigmentations; incontinentia pigmenti achromians (Hypomelanosis of Ito) is a neurocutaneous syndromes characterized by distinctive macular, linear or irregular whorls or swirls of Hypopigmentations. Unrelated to incontinentia pigmenti, the loss of pigment begins spontaneously during infancy or early childhood and is of Particular importance because of the falt that m...
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Background: Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis that presents at the time of birth or soon after birth with cutaneous manifestation. This disorder may also affect the ectodermal tissues, such as the central nervous system, skeletal system, eyes, hair, nails, and teeth. The dermatological findings occur in four successi...
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INTRODUCTION Incontinentia pigmenti is a rare X-linked dominant disease which affects the ectodermal tissues, usually lethal in males. MATERIALS AND METHODS A retrospective analysis of clinical data obtained from the photographic documentation and casenotes of patients diagnosed to have incontinentia pigmenti at the National Skin Centre. The study covered the period from January 1990 to Decem...
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