incontinentia pigmenti: a newborn with characteristic skin lesions and bilateral optic atrophy: case report and review of literature.
نویسندگان
چکیده
incontinentia pigmenti (ip) is a rare x-linked dominant disorder with skin, eye, central nervous system (cns) and tooth abnormalities. according to the reported cases, it is estimated that there have been nearly 900-1200 affected individuals. in this article, the literature is reviewed and a case of ip with characteristic skin lesions and optic atrophy is presented.
منابع مشابه
Incontinentia pigmenti: a newborn with characteristic skin lesions and bilateral optic atrophy: case report and review of literature.
Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder with skin, eye, central nervous system (CNS) and tooth abnormalities. According to the reported cases, it is estimated that there have been nearly 900-1200 affected individuals. In this article, the literature is reviewed and a case of IP with characteristic skin lesions and optic atrophy is presented.
متن کاملIncontinentia pigmenti: a case report and literature review.
Incontinentia pigmenti (IP) is a rare, X-linked dominant disorder that presents at or soon after birth with characteristic cutaneous signs. The eyes and central nervous system are the next most commonly affected systems. We aimed to describe the ophthalmological, neurological and radiodiagnostic findings of a patient with IP and bilateral retinal detachment. Clinical and laboratory findings of ...
متن کاملIncontinentia pigmenti with secondary Raynaud's phenomenon: A case report and review of the literature
Purpose To describe a patient with incontinentia pigmenti (IP) and Raynaud's phenomenon (RP). Observations A 5 year-old girl with history of IP was noted to have RP. Visual acuity was unaffected in both eyes, and fundus examination demonstrated regressed peripheral neovascularization. Photos of the patient's hands demonstrated pale discoloration associated with exposure to cold. Conclusions...
متن کاملIncontinentia pigmenti: a case report.
Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in...
متن کاملA Case Report of Incontinentia Pigmenti in a Newborn with Positive Family History Extending Over Three Generations
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...
متن کاملA Case Report of Incontinentia Pigmenti
Corresponding Author: B. Basiri MD Tel: +98 9188121848 Email: [email protected] Abstract Objective Incontinentia Pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based o...
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عنوان ژورنال:
acta medica iranicaجلد ۵۱، شماره ۱۱، صفحات ۸۰۵-۸۱۰
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