Lymphangioma circumscriptum (LC) is a rare disorder of the lymphatic channels. It can appear at any site. It has been reported to occur in the vulva following radiation therapy (RT)(1–3), though it can also occur in patients without a previous history of irradiation(4,5). It is benign but causes distressing symptoms of itching and oozing of serous fluid in the vulva that has a tendency to get infected.
Here, we report two cases with relevant discussion.
A 30-year-old lady presented to our centre in February 1990, with complaints of foul-smelling discharge through vaginum and burning micturition of 3 months duration. A cervical growth had been biopsied at another hospital and diagnosed as infiltrating squamous cell carcinoma. She was treated with external radiation therapy and intracavitary radiation, delivering a total dose of 70 Gy to point A. Total dose to the skin ranged from 38 to 45 Gy.
After this, the patient was on regular follow up. Patient was disease free till July 1992, when she presented with subcutaneous edema of abdominal wall in hypogastric region, pubic region, and labia majora, with severe itching of the abdominal wall and vulva. Her cancer was in remission, but she had chronic lymphedema and skin excoriation. She was given antibiotics and symptomatic treatment with which she became asymptomatic.
In February 2000, she presented with complaints of burning in periurethral region of 1-week duration. On examination, she had multiple vesicular lesions in the labia majora (Fig. 1).
A biopsy of the lesion was taken. Histopathological examination showed sections of skin without subcutis. There was hyperkeratosis and acanthosis of the epidermis. The superficial dermis contained several dilated thin-walled vascular channels lined by a single layer of endothelial cells. The epidermis was focally elevated and thinned over ectatic thin-walled vessels that extended upto the papillary dermis. Mild to moderate mononuclear inflammatory cell infiltrate in the superficial dermis and around blood vessels and adnexal structures in the dermis was seen. There was no evidence of malignancy. The impression was that of LC.
The patient was placed on monthly long-acting penicillin (Penidure) prophylaxis to prevent infection. She defaulted for prophylaxis for 6 months, then came in November 2002 with cellulitis of lower abdominal wall and was admitted and treated with antibiotics and symptomatic care.
She is now symptom free with regular doses of long-acting Penicillin (Penidure).
A 45-year-old lady, a known case of carcinoma cervix, in remission, presented to us in September 2001 for a routine check up. In the past, she had been treated elsewhere for carcinoma of the cervix. She had been given external radiation therapy to the pelvis up to a dose of 60 Gy. She had completed treatment in February 2000. When she presented to us in September 2001, she was disease free. She presented again in July 2003 with complaints of small growths in vulva. She was seen by the dermatologist, and a skin biopsy was taken. It showed classical features of a lymphangioma circumscriptum with ectatic lymphatics producing attenuation of the epidermis, seen in the upper papillary and reticular dermis (Fig. 2). There was no evidence of malignancy. As the lesions were very small and the patient did not have any discomfort attributable to it, she was advised follow up.
The pathology in LC involves altered lymphatics that form lymphatic cisterns which communicate with the skin and form vesicles. The lymph oozes from these vesicles and causes itching and secondary infection that can be very distressing for the patient.
An exhaustive literature review done by Vlastos et al.(6) reports that only 20 cases of the acquired form of this disease have been reported, of whom 11 had occurred following RT. They have reviewed cases up to 2001 and following that there have been two more reports, summarized in Table 1.
Carbon dioxide laser treatment has been advocated for these patients(7). This is expensive, requires expertise, and is not freely available in developing countries. Recurrences are known to occur even with this treatment. We chose to give protracted long-acting penicillin (Penidure) injections for our patient with the aim of reducing secondary infection and also because it was inexpensive. Patient compliance was reasonable and symptom relief acceptable.
When it occurs in previously irradiated patients, it may be mistaken for recurrence of malignancy or genital warts, and a high degree of clinical suspicion is necessary to make a diagnosis of this disease that is amenable to treatment.
1 La Polla J, Foucar E, Leslin B, et al.
Vulvar lymphangioma circumscriptum; a rare complication of therapy for squamous cell carcinoma of cervix. Gynecol Oncol
2 Schwab RA, McCollough ML et al.
Acquired vulvar lymphangiomas: a sequel of radiation therapy. Cutis
3 Jappe U, Zimmermann T, Kahle B et al.
Lymphangioma circumscriptum of the vulva following surgical and radiological therapy of cervical cancer. Sex Transm Dis
4 Abu-Hamad A, Provencher D, Ganjei P et al.
Lymphangioma circumscriptum of the vulva: case report and review of literature. Obstet Gynecol
5 Esquivias Gomez JI, Miranda Romero A, Cuarado Valles C et al.
Lymphangioma circumscriptum of the vulva. Cutis
6 Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of literature. Obstet Gynecol
7 Huiligol SC, Neill S, Barlow RJ. CO2
laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surg