Lymphangioma circumscriptum in an adult, blood-tinged.

LYMPHANGIOMAS and LYMPHANGIOMA CIRCUMSCRIPTUM

Just as blood vessels may dilate, forming hemangiomas, lymphatic vessels may dilate, forming lymphangiomas.

Lymphangioma circumscriptum is a congenital (or soon thereafter) lymphatic malformation of the skin appearing as a group of fluid-filled lymphatic vesicles or blebs. Ninety percent are detected in the first two years of life. The fluid is lymphatic and clear or blood-tinged.

Lymphangiomas may also occur later in life on the face, on the chest after mastectomy and radiation therapy or in any location in the setting of chronic lymphedema, e.g. the groin after surgery.

Clinical of Lymphangioma Circumscriptum

Grouped vesicles with onset from birth to adulthood is characteristic of lymphangioma circumscriptum. It may be congenital (90% onset first two years of life) and large or acquired and small. The tongue is a common site. Oozing, crusting, swelling, pain and itch are not uncommon. The fluid may be blood-tinged or show a blood/fluid line. These lesions often communicate with deeper lymphatics which may explain why simple destruction commonly leads to recurrence.

Treatment

No treatment is necessary. Simple destruction , e.g. electrodessication or surgical excision may be tried. The fractional 10,600-nm carbon dioxide (CO2) laser and if the fluid is blood tinged, the tunable dye laser have been used.

Sirolimus

Topical sirolimus may be of significant benefit in lymphatic vascular abnormalities in children.

The commercially available oral siroliums solution 0.1% may be used, applied once or twice daily.
Systemic absorption is negligible with blood levels being undetectible.
Response is relatively rapid, within the first 3 months.
Patients may benefit from decreased leakage, shrinkage in the size of the lesion or both.

A simple lymphangioma which is a blister or bleb of the lymphatics.

Blood-tinged lymphangiomas after mastectonmy.

Lymphangiomas of the scrotum in the setting of chronic lymphedema.

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