Background: Lymphatic malformation, a benign malformation of the skin and the subcutaneous tissues, is divided into two major groups: the classical and the localized forms. Pathologically lymphatic malformation often consists of sequestered lymphatic cisterns with thick muscle walls lying deeply in the subcutaneous tissue. Communicating via dermal lymphatic channels with superficial pseudovesicles, they can vary in size depending on the pressure transmitted by the cisterns beneath.
Methods: We present a patient with mixed capillary/lymphatic malformation and coexisting port-wine stain since birth. To demonstrate the anatomic extent and the subcutaneous involvement we performed a 3D reconstruction of a magnetic resonance imaging (MRI). The diagnostic procedures, therapeutic possibilities, and complications regarding this rare appearance are reviewed.
Results: Good results could be obtained with CO2 laser vaporization of the superficial lesions and computed tomography (CT)-guided transcutaneous sclerotherapy for the deeper cisterns with doxycycline.
Conclusion: The combination of CO2 laser treatment and sclerotherapy with doxycycline seems to present a treatment option for cutaneous and subcutaneous lymphangioma circumscriptum with rare side effects.