Infantile hemangioma - Anogenital in
Only about 1%-2% of hemangiomas involve the genitalia. Such hemangiomas of the urethra and, in girls, the labia, hymen, or peri-hymenal area may incorrectly cause concern for abuse.
Ulceration occurs in about 5% of hemangiomas, most commonly in the anogenital region. Ulcerated hemangiomas are painful, often become secondarily infected, and leave a scar. Bleeding and ulceration can incorrectly cause concern for possible abuse. However, their characteristic development in early infancy and typical regression should help differentiate them from abuse. Vulvar hemangiomas with extensive ulcerative changes have been confused with perineal burns secondary to child abuse. Knowledge of the clinical appearance and natural course of untreated hemangiomas may help prevent misdiagnosis.
Regional infantile hemangiomas involving the anogenital area may represent one aspect of associated anomalies designated as LUMBAR (Lower body hemangioma, lipoma or other cutaneous anomalies, Urogenital anomalies, Myelopathy, Bony deformities, Anorectal and arterial anomalies, Renal anomalies), SACRAL (Spinal dysraphism, Anogenital anomalies, Cutaneous anomalies, Renal and urologic anomalies, associated with angioma of Lumbosacral localization), or PELVIS (Perineal hemangioma, External genitalia malformations, Lipomyelomeningocele, Vesicorenal abnormalities, Imperforate anus, and Skin tag).
D18.01 – Hemangioma of skin and subcutaneous tissue
83343001 – Infantile hemangioma
- Congenital hemangioma (CH) – Fully formed vascular tumors at birth; two main types are rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH), and variants have been reported. The proliferative phase in these tumors occurs in utero, and tumors typically do not proliferate postnatally as seen with infantile hemangiomas. CHs are characterized by hemispheric pink to purple vascular plaques with overlying telangiectasia and the characteristic peripheral rim of pallor. Both RICH and NICH may look similar; however, NICH is less impressive and flatter. Histologically, they are GLUT-1 negative, which helps to differentiate them from other hemangiomas.
- Capillary malformation or "port-wine stain" – Early superficial or segmental infantile hemangiomas, or their precursor lesions, may be confused with port-wine stains, which are capillary malformations present at birth. They are pink or red patches that persist throughout life and do not have the typical growth phase of hemangiomas.
- Sometimes tufted angioma and kaposiform hemangioendothelioma may mimic hemangiomas. They present as extensive brownish vascular plaques with deep fibrotic consistency.
- Infantile myofibromatosis – Fibrohistiocytic proliferations characterized by skin-colored to purple, firm to hard or rubbery nodules.
- Deep hemangiomas sometimes look similar to venous malformations, which do not go through a phase of rapid proliferation. Phleboliths are often seen in venous malformations. Bedside Doppler study or ultrasound imaging can help to differentiate these two entities.
- Cystic schwannoma
- Fibrous histiocytoma
- Vascular leiomyoma
- Kaposi sarcoma
Last Updated: 09/04/2018