Sirolimus-associated scrotal lymphedema and secondary development of lymphangiomatosis: Successful treatment with shave excision and cauterization

A 56-year-old male renal transplant patient was started on sirolimus as a maintenance immunosuppressant after developing tacrolimus-associated nephrotoxicity. Within a 4.5 year course of taking a mean dose of 4mg sirolimus daily, he progressively sustained severe lower limb, scrotal and penile swelling with numerous multilocular pedunculated papules on the base of his scrotum. This resulted in recurrent cellulitis and grade IV haemorrhoids, causing significant discomfort. Venous Doppler ultrasound of the pelvis and lower limbs, CT abdomen and pelvis, and whole-body bone scan ruled out venous, infective, obstructive or neoplastic aetiologies of lymphedema. Lymphoscintigraphy showed abnormal lymphatic drainage in both legs. Sirolimus was subsequently discontinued and he was commenced on regular manual lymphatic drainage, daily compression stockings, support jock, and long-term prophylactic antibiotic therapy. Despite the cessation of sirolimus, there was progression of his lymphedema over the last 6 years. Shave excisions of the scrotal papules confirmed a diagnosis of lymphangiomatosis. Given the significant ooze and malodour from the scrotal area, three sessions of shave biopsies and cauterization were performed under local anaesthesia. Complete healing was achieved within one week of each session without further scrotal cellulitis. He had minimal lymphatic leakage from the treated areas of the scrotum. Argon and carbon dioxide (CO2) laser, cutting diathermy, electrocautery, cryotherapy and surgical excision have been used as treatment modalities for acquired lymphangiomas but recurrence is common. For a patient with significant anaesthetic and surgical risk, shave biopsies can be a safe and effective alternative in treating lymphangiomas.

Dr. Minhee Kim