OBSOLETE: Circumscribed lymphatic malformation

Circumscribed lymphatic malformation, also known as lymphangioma circumscriptum, is a type of lymphatic malformation characterized by clusters of small, fluid-filled vesicles (blisters) on the skin surface. This condition has been classified as an obsolete entity in Orphanet (code 217410), meaning it has been reclassified or merged into broader categories of lymphatic malformations. The term historically referred to a localized form of lymphatic malformation that primarily affects the skin, presenting as grouped, translucent or blood-tinged vesicles that resemble frog spawn. These lesions most commonly appear on the proximal limbs, trunk, axillae, and oral mucosa. The underlying abnormality involves dilated lymphatic channels in the superficial dermis that communicate with deeper cistern-like lymphatic reservoirs in the subcutaneous tissue. Patients may experience intermittent leakage of lymphatic fluid from the vesicles, recurrent infections (cellulitis), and cosmetic concerns. The condition is typically present at birth or develops during early childhood, though it can occasionally appear later in life. Treatment options include surgical excision (which must address the deeper lymphatic cisterns to prevent recurrence), sclerotherapy, laser therapy (such as CO2 laser ablation), and radiofrequency ablation. Recurrence rates are relatively high, particularly when deeper components are not adequately addressed. Current classification systems now generally categorize this condition under the broader umbrella of lymphatic malformations, consistent with the International Society for the Study of Vascular Anomalies (ISSVA) classification framework.

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