Sparing of the nipple-areola complex by capillary malformations: Vascular variant of the Bork-Baykal phenomenon

Pediatr Dermatol. 2019 Jul;36(4):558-560. doi: 10.1111/pde.13844. Epub 2019 Apr 15.

Abstract

The"Bork-Baykal phenomenon" refers to the sparing of the nipple-areola complex in large congenital melanocytic nevi involving the breast. So far, this finding has not been reported in vascular anomalies. We present four patients with an extensive capillary malformation (CM) involving the breast that was found to exhibit a similar sparing of the nipple and areola. All of these capillary nevi were associated with asymmetric overgrowth.

Keywords: Bork-Baykal phenomenon; PIK3CA-related overgrowth spectrum; capillary malformation; megalencephaly-capillary malformation; reticulate capillary malformation; reticulate capillary nevus.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Capillaries / abnormalities*
  • Child
  • Class I Phosphatidylinositol 3-Kinases / genetics*
  • Diagnosis, Differential
  • Female
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Infant
  • Magnetic Resonance Imaging / methods
  • Male
  • Nevus, Pigmented / genetics
  • Nevus, Pigmented / pathology*
  • Nevus, Pigmented / physiopathology
  • Nipples
  • Sampling Studies
  • Severity of Illness Index
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / physiopathology
  • Vascular Malformations / diagnosis*
  • Vascular Malformations / genetics*

Substances

  • Class I Phosphatidylinositol 3-Kinases
  • PIK3CA protein, human

Supplementary concepts

  • Capillary Malformations, Congenital, 1