Extensive and invasive lymphangioma circumscriptum in a young female: A rare case report and review of the literature
A 21-year-old female presented to the dermatology out-patient department with multiple hemorrhagic vesicles over the right side of the abdomen, buttock and thighs since birth. Immediately after birth, bluish-colored red plaque was noticed by her mother in the anterior aspect of the right thigh. Gradually, over the next 6 months, multiple water-filled lesions appeared over the whole of the right buttock and right side of the lower abdomen. It was associated with recurrent episodes of pain and fever. At 1 year of age, she was taken to a plastic surgeon and the whole lesion was excised under general anesthesia. After 1 year, the lesions recurred with greater involvement of the thighs, buttocks and right lower abdomen. At 5 years of age, magnetic resonance imaging (MRI) scan showed extensive and deep LC with involvement of deep fascia, muscles of abdomen and buttocks and large vessels, including right iliac and right femoral arteries. The new fluid-filled vesicles appeared over the thighs and legs as well. There was history of episodes of bleeding from the lesion associated with pain. After the MRI scan, she was advised for rest and non-surgical management of the LC as it was deep and extensive.\nExamination revealed a 20 cm X 15 cm-sized large area of hemorrhagic fluid-filled vesicles of varying size from 3 mm to 1 cm over the right lower abdomen and buttocks and extending down toward the right thigh [Figures and ]. Few scattered clusters of vesicles were also seen over the posterior aspect of the right thigh []. The diameter of the right thigh was 6 cm more than that of the left one. Areas of hemorrhage were seen on the lesion. The area was non-tender and there was no rise in local temperature. The fluctuation, compression and cough impulse were absent. No bruit/thrill was present. All other blood investigations like complete blood count, liver function and renal functions were within normal limits.\nHistopathology of a vesicle showed irregular acanthosis of the epidermis, dilated lymphatics lined by endothelial cells in the dermis and containing numerous erythrocytes, macrophages and lymphocytes within the lumen [Figures and ].\nThe patient was referred to the department of plastic surgery for cosmetic debulking of the tumor. She was also reassured regarding the benign nature of the tumor.