Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report
نویسندگان
چکیده
منابع مشابه
Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report
Incontinentia pigmenti (IP; Bloch-Sulzberger syndrome) is a rare, genetic syndrome inherited as an X-linked dominant trait. It primarily affects female infants and is lethal in the majority of males during fetal life. The clinical findings include skin lesions, developmental defects, and defects of the eyes, teeth, skeletal system, and central nervous system. Cardiovascular complications of thi...
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
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...
متن کاملDental defects in incontinentia pigmenti: case report.
Incontinentia pigmenti is an uncommon type of ectodermal dyspIasia involving abnormalities of the skin, hair, central nervous system, and teeth. The literature is reviewed and a case with a variety of dental abnormalities is presented. Incontinentia pigrnenti (IP) is one of the lesser known ectodermal dysplasias with fewer than 800 cases reported in the world literature. This genodermatosis aff...
متن کامل[A 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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ژورنال
عنوان ژورنال: Therapeutics and Clinical Risk Management
سال: 2017
ISSN: 1178-203X
DOI: 10.2147/tcrm.s134705