Acquired scrotal lymphangioma circumscriptum following varicocele surgery: A case report and literature review

Dear Editor, Lymphangioma circumscriptum (LC), also called as microcystic lymphatic malformation, is an uncommon hamartoma involving the skin and subcutaneous tissues classically presenting as grouped fluid filled asymptomatic thin walled vesicles resembling frog spawn.The vesicles express clear fluid on rupture, rarely pink/blood tinged in chronic lesions. They are usually congenital, mostly involving proximal limbs, neck, oral cavity, axillary folds, and adjacent areas. Acquired form can develop following local damage to lymphatic channels secondary to infections and inflammation involving lymphatics (filariasis, tuberculous lymphadenitis, donovanosis, and lympho-granuloma-venereum), trauma, surgery, or radiotherapy. Acquired LC involving scrotum is very rare. Lesions are asymptomatic to begin with, but later get infected due to frequent rupture of vesicles and thus the discomfort brings the patient to dermatologist. We present a case of acquired scrotal lymphangioma circumscriptum following varicocele surgery, which has not been reported before. A 60-year-old male presented to us with fluid filled lesions over scrotum for the past 6 years. He noticed skin lesions over scrotum 1 month following varicocele surgery which were progressively increasing in number, some of them rupturing spontaneously with oozing of clear fluid. There was no history suggestive of infections, radiotherapy or any trauma other than varicocele surgery. On cutaneous examination, entire scrotum was found to be studded with vesicles of variable size ranging from 1 to 5 mm in diameter. On rupturing, the vesicles exuded clear fluid. There was no associated lymphadenopathy or lymphedema of lower limbs. A scar was noted over inguinal area on right side, at the root of scrotum (Figure 1). Examination of hair, nails and mucosae was normal. Systemic examination was unremarkable. Routine laboratory tests including filarial serology and serology for sexually transmitted infections were unremarkable. Ultrasonography of the scrotum/groin showed multiple small cysts in the superficial dermis sparing subcutaneous tissues. Skin biopsy showed epidermal hyperkeratosis, acanthosis and elongation of rete ridges. Dermis showed cystic dilated lymphatics with flat endothelial cells in the papillary dermis (Figure 2). Based on history, clinical examination and further supported by ultrasonography and histopathology, the diagnosis of acquired LC was made. Surgical excision was advised, but patient was lost to follow-up. LC also called as localized lymphangiectasia, is a benign cutaneous disorder involving lymphatic dilatations in an area resulting in vesicular/fluid filled lesions due to compromised lymphatic flow. It is usually congenital due to developmental malformations in the superficial lymphatics of skin and underlying tissues. It can also be acquired later in life secondary to disturbed lymphatic flow from various reasons affecting the lymphatics of a particular area. Lymphangioma account for approximately 26% of benign vascular tumors in children. They include LC, cavernous lymphangioma, cystic hygroma, and lymphangioendothelioma. Most common of all is LC which commonly arises in infancy but may occur at any age.