Phakomatosis pigmentovascularis with sturge–Weber syndrome

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Phakomatosis pigmentovascularis type IIa

Phakomatosis Pigmentovascularis (PPV) is a rare cutaneous congenital malformation syndrome, defined as simultaneous occurrence of congenital cutaneous vascular and pigmentary anomalies. As most of the reported cases are from Far East countries, especially Japan, we reported a case of PPV type IIa in an Iranian patient. This case was unusual because of the coexistence of unilateral extensive por...

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Phakomatosis Pigmentovascularis: A case report

An eighteen-year old man is reported who had a large nevus spilus on the right side of his body with port wine stain on the anterior and posterior sides of his trunk without any systemic involvement. Considering the clinical signs and skin biopsy report of the patient, the diagnosis of phakomatosis pigmentovascularis type IIIa was made.

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Bilateral 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...

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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...

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An 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...

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ژورنال

عنوان ژورنال: TNOA Journal of Ophthalmic Science and Research

سال: 2020

ISSN: 2589-4528

DOI: 10.4103/tjosr.tjosr_122_19