Acquired progressive lymphangioma

Acquired progressive lymphangioma, also known as benign lymphangioendothelioma is a group of lymphangiomas that occur anywhere in young individuals, grow slowly, and present as bruise-like lesions or erythematous macules. Acquired progressive lymphangioma may develop following radiation therapy, surgery, trauma, inflammation, and tick bites. The treatment of choice is complete surgical excision.

Signs and symptoms
Acquired progressive lymphangioma appears as a slowly growing erythaematous to brownish, bruise-like, macule, plaque, or nodule typically located on the legs, head or neck. The lesions are usually asymptomatic.

Causes
Acquired progressive lymphangioma has been known to develop after radiation therapy, surgery, trauma, femoral arteriography, inflammation, and tick bites.

Mechanism
Because acquired progressive lymphangioma has been described following various traumas, it is thought to be a response to various inflammatory stimuli rather than a real neoplasm.

Another possible pathogenic explanation is hormonal stimulation, as quickly growing lesions have been observed in numerous pubescent and prepubescent individuals.

Finally, another idea proposed is that acquired progressive lymphangioma represents a complicated vascular hamartoma with three components: lymphatic vessels, blood vessels, and smooth muscle.

Diagnosis
Acquired progressive lymphangioma has thin-walled endothelial-lined gaps interspersed between collagen strands. Endothelial cells show positive staining for lymphatic markers as podoplanin (D2-40), LYVE-1, and PROX-1. Additionally, the cells have variable levels of factor VIII, Ulex europaeus agglutinin I, CD31, and CD34.

Treatment
Acquired progressive lymphangioma is treated by complete surgical excision.