angiomatous nevus; strawberry nevus; capillary hemangioma of infancy

Introduction

Benign vascular proliferation of endothelial lining that develops at birth or soon after birth & disappears by the 5th year of life.

Epidemiology

• most common tumor of infancy
• up to 10% of all births
• 1-2% of Caucasians
• low birth weight is risk factor

Pathology

• benign, highly proliferative lesions involving aberrant localized growth of

localized proliferation of angioblastic mesenchymal tissue (capillary endothelium)

proliferation of endothelial cells in dermis in superficial type, little or no endothelial proliferation in deep type

Genetics

• susceptibility associated with defects in ANTXR1

Clinical manifestations

• bright red (superficial type) to purple (deep type) nodule or plaque 1-8 cm in diameter
• incomplete blanching
• distribution: 50% head & neck, 25% trunk, legs & mucus membranes
• spontaneous involution by 5th year of life
  • no residual skin changes in 80%
  • residual atrophy & depigmentation in 20%
  • deeper lesions & lesions involving mucus membranes may not involute completely
• synovial involvement may be associated with hemophilia-like arthropathy
• large angiomatous nevi
  • may be associated with cavernous hemangiomas
  • may be associated with platelet entrapment & thrombocytopenia (Kasabach-Merritt syndrome)

Diagnostic procedures

• ultrasound as indicated

Radiology

• magnetic resonance imaging for lumbosacral hemangionas > 2.5 cm in diameter identifies spinal abnormalities in 1/2 of the patients

Management

• spontaneous involution is the rule & gives the best long- term cosmetic results
• continuous or pulse dye laser therapy
• cryotherapy with liquid nitrogen
• systemic glucocorticoids

More general terms

• nevus
• cutaneous hemangioma

Additional terms

• cavernous hemangioma (cavernoma, capillary venous lymphatic malformation, CVL, nevus cavernosus, vascular erectile tumor)
• Kasabach-Merritt syndrome

Database

• OMIM: https://mirror.omim.org/entry/602089