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Incontinentia Pigmenti Involving Only the Skin

Md Wahiduzzaman

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Abstract

Incontinentia pigmenti (IP) is an uncommon disorder which can affect the skin and other systems. . It is an X-linked dominant genodermatosis mainly affecting the female baby with prenatal lethality in boys. It was first reported by Garrod in 1906 and further defined by Bloch and Sulzberger in 1926 and 1928. It can involve the skin only or in addition can affect the central nervous system, eyes and the teeth. The boys do not survive unless associated with Klinefelter syndrome (47, XXY). Morbidity and mortality are related to neurologic and ophthalmologic sequelae. Our presented case did not have any systemic involvement but only cutaneous symptoms. doi:10.3329/jom.v10i1.2000 J Medicine 2009; 10: 25-27

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Incontinentia pigmenti (IP) is an uncommon disorder which can affect the skin and other systems. . It is an X-linked dominant genodermatosis mainly affecting the female baby with prenatal lethality in boys. It was first reported by Garrod in 1906 and further defined by Bloch and Sulzberger in 1926 and 1928. It can involve the skin only or in addition can affect the central nervous system, eyes and the teeth. The boys do not survive unless associated with Klinefelter syndrome (47, XXY). Morbidity and mortality are related to neurologic and ophthalmologic sequelae. Our presented case did not have any systemic involvement but only cutaneous symptoms. doi:10.3329/jom.v10i1.2000 J Medicine 2009; 10: 25-27

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Available abstract

Incontinentia pigmenti (IP) is an uncommon disorder which can affect the skin and other systems. . It is an X-linked dominant genodermatosis mainly affecting the female baby with prenatal lethality in boys. It was first reported by Garrod in 1906 and further defined by Bloch and Sulzberger in 1926 and 1928. It can involve the skin only or in addition can affect the central nervous system, eyes and the teeth. The boys do not survive unless associated with Klinefelter syndrome (47, XXY). Morbidity and mortality are related to neurologic and ophthalmologic sequelae. Our presented case did not have any systemic involvement but only cutaneous symptoms. doi:10.3329/jom.v10i1.2000 J Medicine 2009; 10: 25-27

Key concepts: Genodermatosis, Incontinentia pigmenti, Medicine, Dermatology, Affect (linguistics), Pigmentation disorder, Pediatrics, Albinism

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