Case presentation
A 43-year-old man first presented to our dermatology clinic in 2014 with multiple erythematous dome-shaped papules on his right auricle. He has had these lesions from 6 months before his presentation to our clinic (Fig. 1a). A biopsy was taken from his auricular papules at that time. While our most probable clinical impression was Angiolymphoid hyperplasia with eosinophilia (ALHE) or pseudolymphoma, the microscopic evaluation was consistent with KS (Fig. 2a-e). Histopathologic examination of a skin biopsy from the ear showed nodular proliferation of spindled endothelial cells arranged in intersecting fascicles with intervening slit and sieve-like vascular channels. There were some blood-filled vascular spaces between spindle cells with red blood cell extravasation and patchy infiltrate of lymphocytes and plasma cells (Fig. 2a, 2b). Some mitotic figures and apoptotic bodies were also identified. Immunohistochemistry staining reveals positive immunoreaction of tumor cells for CD31 and CD34 as well as HHV-8 which show nuclear immunoreactivity (Fig. 2c-e).
Because his lesions were limited to his ear, the lesions were totally excised (Fig. 1b). In 2018, he presented to our clinics with recurrence of one solitary papule on his right ear, the papule was totally excised and the histopathology was consistent with KS again. The patient did not come back for further evaluation at that time. In April 2020, he presented to our clinic with the recurrence of papules on his right ear and the development of an erythematous plaque on his right foot since a year ago. Two biopsies were taken from his ear and foot lesions that both were consistent with KS. Routine laboratory evaluations including complete blood count (CBC), liver and renal function tests were normal and HIV testing was negative. The patient was otherwise healthy without any history of immunodeficiency. He was not taking any medication.