Cutaneous Lymphangioma Circumscriptum
A 12-year-old boy presented with an asymptomatic eruption over the right cheek, which first had been noticed 3 years previously. On physical examination, a cluster of 2- to 3-mm, light brown, translucent vesicles resembling frogspawn was present over the right cheek (A).
A skin biopsy was obtained and submitted for hematoxylin and eosin staining. Biopsy results at ×400 magnification showed mild hyperkeratosis with numerous grouped, dilated, cystic lymphatic spaces in the papillary dermis. The cisterns were lined with flattened endothelium, with some filled with lymphatic fluid and few red and white blood cells (B). Magnetic resonance imaging revealed the presence of deep cisterns extending to the subcutaneous tissue. The child received a diagnosis of cutaneous lymphangioma circumscriptum (CLC) of the face. He was referred to a plastic surgeon for complete excision.
CLC belongs to the group of lymphangiomas that are hamartomatous, congenital malformations of the lymphatic system involving the skin and subcutaneous tissues. Lymphangiomas are uncommon, accounting for 4% of all vascular tumors and about 25% of all benign vascular tumors in children.
The probable pathogenesis of CLC involves the growth of abnormal lymphatic cisterns separately from normal lymph vessels in the deep subcutaneous tissue. Dilation of these ectopic lymphatics due to smooth muscle contractions results in protrusions from the skin in the form of translucent vesicles. The proximal extremities, trunk, axillae, and tongue are more commonly involved, but lesions may occur anywhere, including the genitalia.
Two subtypes of CLC have been described: classic, with an onset soon after birth, a predilection for specific sites, and a size usually larger than 1 cm2; and localized, with onset at any age and any site and a size usually smaller than 1 cm2. Differential diagnosis includes lymphangiectasia, hemangioma, angiokeratoma, lymphangioendothelioma, verrucae, and molluscum contagiosum.
The treatment of choice is complete surgical excision through the full thickness of skin down to the deep fascia until normal subcutaneous tissue has been exposed. Other treatments have been tried, including cryotherapy, sclerotherapy, cauterization, laser ablation, and intralesional bleomycin, but recurrence is least likely with complete surgical excision.
Deepak M. Kamat, MD, PhD—Series Editor:Dr Kamat is professor of pediatrics at Wayne State University in Detroit. He is also director of the Institute of Medical Education and vice chair of education at Children’s Hospital of Michigan, both in Detroit.