PHACES syndrome presenting as hemangiomas, sternal clefting and congenital ulcerations on the helices

Durusoy G., Mihci E., Tacoy S., Ozaydin E., Alpsoy E.

JOURNAL OF DERMATOLOGY, cilt.33, sa.3, ss.219-222, 2006 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 33 Sayı: 3
  • Basım Tarihi: 2006
  • Doi Numarası: 10.1111/j.1346-8138.2006.00050.x
  • Dergi Adı: JOURNAL OF DERMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.219-222
  • Anahtar Kelimeler: hemangioma, PHACES syndrome, sternal malformation/vascular dysplasia association
  • Akdeniz Üniversitesi Adresli: Evet

Özet

Sternal malformation/vascular dysplasia association is a very rare condition comprised of midline defects and hemangiomas of the face and anterior trunk, that can be found as part of the PHACES phenotypic spectrum (posterior fossa malformations, hemangiomas, arterial anomalies, coarctations of the aorta, cardiac defects and eye abnormalities, sternal clefting). Herein, we describe a 6-month-old boy with sternal cleft, extensive segmental hemangiomas, and a depigmented scar on the tip of the xyphoid process, corresponding to a sternal malformation/vascular dysplasia association. He also had bilateral cutaneous ulcerations on the helices. Our case report indicates that ulceration of a hemangioma can occur before significant proliferation and may even be present congenitally.