Phakomatosis pigmentovascularis type IIB in association with external hydrocephalus

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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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ژورنال

عنوان ژورنال: Case Reports

سال: 2012

ISSN: 1757-790X

DOI: 10.1136/bcr.12.2011.5432