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angiomatous nevus; strawberry nevus; capillary hemangioma of infancy

Benign vascular proliferation of endothelial lining that develops at birth or soon after birth & disappears by the 5th year of life. Epidemiology: 1) most common tumor of infancy 2) up to 10% of all births 3) 1-2% of Caucasians 4) 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: 1) bright red (superficial type) to purple (deep type) nodule or plaque 1-8 cm in diameter 2) incomplete blanching 3) distribution: 50% head & neck, 25% trunk, legs & mucus membranes 4) spontaneous involution by 5th year of life a) no residual skin changes in 80% b) residual atrophy & depigmentation in 20% c) deeper lesions & lesions involving mucus membranes may not involute completely 5) synovial involvement may be associated with hemophilia-like arthropathy 6) large angiomatous nevi a) may be associated with cavernous hemangiomas b) may be associated with platelet entrapment & thrombocytopenia (Kasabach-Merritt syndrome) Special laboratory: - ultrasound as indicated Radiology: - magnetic resonance imaging for lumbosacral hemangionas > 2.5 cm in diameter identifies spinal abnormalities in 1/2 of the patients [2] Management: 1) spontaneous involution is the rule & gives the best long- term cosmetic results 2) continuous or pulse dye laser therapy 3) cryotherapy with liquid nitrogen 4) systemic glucocorticoids

Related

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

General

nevus cutaneous hemangioma

Database Correlations

OMIM 602089

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 149-150
  2. Drolet BA et al. Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin. J Pediatr 2010 Nov; 157:789. PMID: 20828712