PT  - JOURNAL ARTICLE
AU  - Peter Okunola
AU  - Gabriel Ofovwe
AU  - Moses Abiodun
AU  - Abiodun Isah
AU  - Joyce Ikubor
TI  - Phakomatosis pigmentovascularis type IIB in association with external hydrocephalus
AID  - 10.1136/bcr.12.2011.5432
DP  - 2012 Jun 26
TA  - BMJ Case Reports
PG  - bcr1220115432
VI  - 2012
4099  - http://casereports.bmj.com/content/2012/bcr.12.2011.5432.short
4100  - http://casereports.bmj.com/content/2012/bcr.12.2011.5432.full
AB  - Phakomatosis pigmentovascularis (PPV) is a rare sporadic genetic disorder characterised by co-occurrence of an extensive vascular nevus and a large pigmentary nevus with or without extracutaneous manifestations. There are four types of PPV with subtype ‘a’ for cutaneous involvement only and subtype ‘b’ for cutaneous and systemic involvement. PPV type IIa consists of nevus flammeus, Mongolian spots and sometimes nevus anemicus. Prognosis depends on associated systemic disorders. Two independent cases of PPV type IIb presented with nevus flammeus, aberrant Mongolian spots, ocular and central nervous system anomalies. Case 1 had external hydrocephalus previously unreported in PPV while case 2 had hydrocephalus exvacuo. Both patients had seizure disorder and neurodevelopmental delay. They were on long-term neurologic and ophthalmologic management while their cutaneous lesions partially regressed. PPV affects all racial and ethnic groups. The occurrence of external hydrocephalus in PPV expands the spectrum of its systemic manifestations.