An Unusual Case of Lymphangioma-Like Kaposi Sarcoma in a Kidney Transplant Patient

Introduction: Lymphangioma-like Kaposi sarcoma (LLKS) is a rare histologic presentation of Kaposi sarcoma (KS). LLKS has been described in acquired immunodeficiency syndrome and in endemic African-type as well as classic indolent KS. Here we present a rare case of transplant-associated iatrogenic immunosuppression–induced LLKS Case Description: 71 yrs old Caucasian male with PMH of ESRD 2/2 PKD who underwent DDKT, presented with skin lesions at 1 yr post-transplant follow-up. He initially noticed the lesion around the time of transplantation as a bruise-like spot on his right foot and over the past one year gradually developed numerous skin lesions affecting multiple sites. HHV8-pcr was positive. His immunosuppressant at that time comprised of tacrolimus 2mg bid and MMF 1250mg bid. Dermatology referral was done who described the lesion as violaceous discoloration and edema with erythematous patches, ill-defined vascular plaque, purplish-blue papules and nodules, with edema and induration of right foot and lower leg. Skin biopsy revealed grossly dilated and anastomosed vascular channels with prominent hobnail endothelial cells, adjacent stroma showed fibroplasia with a proliferation of atypical spindled-shaped cells, numerous erythrocytes in the adjacent stroma as well as mononuclear cells and plasma cells. Immunohistochemistry was positive for HHV-8 and podoplanin. C-MYC was negative. Tacrolimus was changed to everolimus with improvement and stabilization of the Kaposi's lesions Discussion: LLKS is a rare histologic presentation of KS which is strongly associated with HHV-8 infection. Incidence of Kaposi sarcoma in renal transplant is 1.5%, however the incidence of LLKS in renal transplant patient is extremely rare. mTOR inhibits the HHV-8 lytic replication cycle and has an antineoplastic and antiangiogenic effects. This case report represents further evidence of the utility of an mTOR inhibitor as a treatment for kidney transplant–associated iatrogenic immunosuppression–induced Kaposi sarcoma