Lymphangioma circumscriptum

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Lymphangioma circumscriptum
Lymphangioma circumscriptum on the skin
Synonyms Microcystic lymphatic malformation
Pronounce N/A
Specialty N/A
Symptoms Clusters of small, blister-like lesions
Complications Infection, bleeding
Onset Congenital or early childhood
Duration Long-term
Types N/A
Causes Malformation of the lymphatic system
Risks None specific
Diagnosis Clinical diagnosis, biopsy
Differential diagnosis Hemangioma, molluscum contagiosum, warts
Prevention N/A
Treatment Laser therapy, surgical excision
Medication None specific
Prognosis N/A
Frequency Rare
Deaths N/A


Lymphangioma circumscriptum is a benign lymphatic malformation characterized by clusters of small, translucent vesicles on the skin. These vesicles are often described as resembling frog spawn or tapioca pudding. Lymphangioma circumscriptum is a type of lymphangioma, which is a broader category of malformations involving the lymphatic vessels.

Pathophysiology

Lymphangioma circumscriptum occurs due to the abnormal development of the lymphatic system. It is thought to arise from a congenital malformation of the lymphatic vessels, leading to the formation of dilated lymphatic channels in the superficial dermis. These channels become filled with lymph fluid, resulting in the characteristic vesicular appearance on the skin.

Clinical Presentation

The condition typically presents as clusters of small, clear, or pinkish vesicles on the skin. These vesicles can vary in size and may be associated with underlying subcutaneous swelling. Lymphangioma circumscriptum most commonly appears on the proximal extremities, trunk, and axillary regions, but it can occur anywhere on the body.

Diagnosis

Diagnosis of lymphangioma circumscriptum is primarily clinical, based on the appearance of the lesions. A biopsy may be performed to confirm the diagnosis and rule out other conditions. Histologically, the lesions show dilated lymphatic channels in the dermis, lined by a single layer of endothelial cells.

Treatment

Treatment options for lymphangioma circumscriptum include surgical excision, laser therapy, and sclerotherapy. The choice of treatment depends on the size, location, and symptoms of the lesions. Complete surgical excision is often curative but may not be feasible for extensive lesions. Laser therapy can reduce the appearance of the vesicles, while sclerotherapy involves injecting a sclerosing agent to shrink the lymphatic channels.

Prognosis

Lymphangioma circumscriptum is a benign condition and does not typically pose a significant health risk. However, it can cause cosmetic concerns and may be associated with recurrent infections or bleeding if the vesicles are traumatized. Recurrence after treatment is possible, especially if the lesions are not completely excised.

See also

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Contributors: Prab R. Tumpati, MD