Phakomatosis Pigmentovascularis Associated With Sturge–Weber Syndrome, Ota Nevus, and Congenital Glaucoma
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چکیده
منابع مشابه
Phakomatosis Pigmentovascularis Associated With Sturge–Weber Syndrome, Ota Nevus, and Congenital Glaucoma
Phakomatosis pigmentovascularis (PPV) is a rare congenital malformation syndrome that is characterized by a combination of capillary abnormalities and dermal melanocytosis.We describe 3 cases of PPV combined with bilateral Sturge-Weber syndrome (SWS), Ota nevus, and congenital glaucoma.Case 1 was a 2-year-old boy. Facial port-wine stains distributed along the 3 branches of his trigeminal nerves...
متن کاملBilateral Sturge-Weber and Phakomatosis Pigmentovascularis with Glaucoma, an Overlap Syndrome
UNLABELLED Aim. To report a case of bilateral Sturge-Weber and Phakomatosis pigmentovascularis with secondary glaucoma in a child. Method. CASE REPORT Results. A 4-year-old male child was referred to us for control of intraocular pressure (IOP). Sleeping IOP was 36 mm Hg in right eye and 28 mm Hg in the left eye. The sclera of both the eyes showed bluish black pigmentation-melanosis bulbi. Fu...
متن کاملAn Infantile Case of Sturge-Weber Syndrome in Association with Klippel-Trenaunay-Weber Syndrome and Phakomatosis Pigmentovascularis
Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a...
متن کاملReport of a case of Klippel-Trenaunay syndrome associated with bilateral nevus of Ota
Port-wine stain is a vascular malformation characterized pathologically by ectasia of superficial dermal capillaris and clinically by persistent macular erythema. The association of a port-wine stain on a limb with soft tissue swelling, with or without bony overgrowth, is termed klippel-Trenaunay syndrome. Phakomatosis pigmento-vascularis is a combination of port-wine stain and cutaneous ...
متن کاملBilateral congenital nevus of Ota in association with Mongolian spot
A 24-year-old woman presented with asymptomatic hyperpigmented bilateral patches on her temples, eyelids and forehead since birth. Furthermore, the patient had a congenital grey patch, compatible with Mongolian spot, on her buttock. She had no vascular or other cutaneous lesion. Histopathologic examination revealed bipolar dendritic melanocytes dispersed in a ribbon-like pattern between the col...
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ژورنال
عنوان ژورنال: Medicine
سال: 2015
ISSN: 0025-7974
DOI: 10.1097/md.0000000000001025