Lymphangioma Circumscriptum With Secondary Cellulitis

For 3 days, a 6-year-old boy had “redness and pain” of skin on his left upper abdomen. Physical examination revealed a large cluster of vesicles with underlying erythema and mild warmth. The patient’s mother stated that the lesion had been present for 5 years, that it had been diagnosed as lymphangioma circumscriptum by a dermatologist, and that excision was pending. Secondary cellulitis of the existing lesion was considered, and an oral antibiotic was prescribed. At follow-up, signs of infection had cleared.

Lymphangiomas, which make up about 4% of all vascular tumors and 26% of benign vascular tumors in children, are divided into 4 main types: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, and benign lymphangioendothelioma.¹ Lymphangioma circumscriptum is a rare type caused by dilation of subcutaneous lymphatic cisterns, which leads to outpouchings that protrude through the skin and form vesicles.² Half of cases present at birth, 90% by age 2 years.³

Clinically, the disorder can be described as clusters of small (usually 1- to 2-mm) translucent vesicles; hemorrhage in and around the vesicles may cause the lesions to appear pink, red, or black.² The lesions can occur anywhere on the skin or mucous membranes, but they most commonly develop on the proximal extremities, trunk, axillae, and buttocks.¹ The differential diagnosis can include warts, molluscum contagiosum, hemangioma, angiokeratoma, lymphangioendothelioma, lymphangiectasis, metastatic carcinoma of the skin, herpes simplex, herpes zoster, dermatitis herpetiformis, and malignant melanoma.^2,3

Treatment may be considered for cosmesis, pain, swelling, and recurrent cellulitic infections.³ Options include cryotherapy, laser therapy, sclerosing agents, and suction-assisted lipectomy; however, surgical resection using MRI to obtain adequate margins is the preferred method.²

References:

REFERENCES:

1. Heller M, Mengden S. Lymphangioma circumscriptum. Dermatol Online J. 2008;14:27.

2. Mordehai J, Kurzbart E, Shinhar D, et al. Lymphangioma circumscriptum. Pediatr Surg Int. 1998;13:208-210.

3. Patel GA, Schwartz RA. Cutaneous lymphangioma circumscriptum: frog spawn on the skin. Int J Dermatol. 2009;48:1290-1295.