Lymphangioma

Lymphangioma

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Key Points
*Congenital skin condition which is a type of birthmark
*Exact cause is unknown, but occurs as a result of lymphatic system malformations
*Symptoms dependent on what type of the condition is extant

Lymphangioma is a congenital skin condition which is a type of birthmark. Cutaneous symptoms may be very small or large enough to affect an entire limb. It presents in three distinct types, cystic hygromas, cavernous lymphangioma, and lymphangioma circumscriptum. Cystic hygroma consists of large spaces filled with fluid resembling swelling under the skin, which may be transparent and red, blue or skin colored. These lesions typically appear on the armpits, neck and groin, and may be vulnerable to secondary infection. Cystic hygroma is often found in association with other congenital conditions such as Down syndrome, fetal alcohol syndrome, Turner syndrome, or hydrops fetalis.

Cavernous lymphangioma consists of red, blue or skin colored swelling under the skin, which is rubbery in consistency. Lesions may appear anywhere on the body, and may see periods of rapid growth in very young children. In rare cases lesions may ulcerate. Lymphangioma circumscriptum consists of small blisters which are firm and filled with fluid, and may be pink, red, brown or black in color. Lesions may develop a warty texture, and are most commonly found on the neck, limbs, underarm, neck, mouth and tongue. Lesions may become more pronounced during puberty, and can leak fluid and/or bleed. Lesions rarely may be vulnerable to secondary infection.

The exact cause of lymphangioma is unknown, but the condition occurs as a result of abnormalities in the development of the lymphatic system, in which disconnected lymphatic vessels in the skin are malformed.

Differential Diagnosis (Other conditions with similar appearance)
Dabska Tumor
Malignant Melanoma
Dermatitis Herpetiformis
Metastatic Carcinoma of the Skin
Herpes simplex
Neurofibromatosis
Herpes Zoster
Stewart-Treves Syndrome
Lipomas
Lymphangiectasia

Diagnosis
Key Points
*Diagnosis based on appearance of the affected area
*Biopsy and other testing may be performed to confirm diagnosis and rule out other conditions

Lymphangioma is typically diagnosed based on the appearance of the affected area. A biopsy will confirm the diagnosis and rule out other conditions, and imaging tests may be performed to determine the extent of the condition.

Treatment
*Treatment typically consists of removal of lesions
*Lymphangioma circumscriptum does not generally require treatment
*Antibiotics may be administered to treat secondary infection

Treatment of cystic hygroma and cavernous lymphangioma typically consists of removal of the lesions by surgical excision, though if lesions are deeply seated it is difficult to fully excise them, which may result in recurrence of the lesion. Lymphangioma circumscriptum does not generally require treatment, as the associated lesions are harmless. If necessary, these lesions may also be removed, but are also subject to recurrence. In all cases where it occurs, secondary infections will be treated with antibiotic medications.

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