Case reports

Dermatology
Volume 195 - Issue 11

Birthmarks: phacomatosis pigmentovascularis

Authors:  Jianjun Qiao and Hong Fang

Med J Aust 2011; 195 (11): 709. || doi: 10.5694/mja11.10720
Published online: 12 December 2011
Download PDF (73 KB)
Share: Share on Blue Sky Share on Facebook Share on LinkedIn Share by email

A 36-year-old Chinese man had been born with multiple coloured lesions on his face, sclera, neck, trunk and limbs. Physical examination revealed extensive capillary malformations (naevus flammeus or port wine stain) on his neck, chest, back and upper limbs (Figure, A). Bluish-grey macules involved the left V2 dermatomal distributions of his face and conjunctiva, consistent with naevus of Ota and melanosis oculi (Figure, A). Aberrant Mongolian spots were present on his trunk (Figure, B and C). No other positive findings were noted. A diagnosis of phacomatosis pigmentovascularis (phacomatosis cesioflammea form) was made.

Phacomatosis pigmentovascularis is defined as the coexistence of an extensive vascular naevus and various melanocytic lesions.1 It is estimated that 50% of patients with phacomatosis pigmentovascularis have systemic involvement, particularly of the eyes and central nervous system.1,2 No systemic involvement was evident in this patient.

Authors

  • Jianjun Qiao1
  • Hong Fang2
  • 1. The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China.

Correspondence: fanghongzy@sina.com

References

View this article on Wiley Online Library

More like this

View more