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Italian Journal of Dermatology and Venereology 2021 June;156(3):356-65

DOI: 10.23736/S2784-8671.20.06703-6

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Diagnosis and treatment of classic and iatrogenic Kaposi’s sarcoma: Italian recommendations

Lucia BRAMBILLA ¹, Giovanni GENOVESE ^{1, 2}, Emilio BERTI ^{1, 2}, Ketty PERIS ^{3, 4}, Franco RONGIOLETTI ⁵, Giuseppe MICALI ⁶, Fabio AYALA ⁷, Silvia DELLA BELLA ^{8, 9}, Roberta MANCUSO ¹⁰, Piergiacomo CALZAVARA PINTON ¹¹, Athanasia TOURLAKI ¹ ✉

¹ Unit of Dermatology, Maggiore Polyclinic Hospital, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; ² Department of Medical-Surgical Pathophysiology and Transplantation, University of Milan, Milan, Italy; ³ Unit of Dermatology, IRCCS A. Gemelli University Polyclinic Foundation, Rome, Italy; ⁴ Unit of Dermatology, Sacred Heart Catholic University, Rome, Italy; ⁵ Unit of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy; ⁶ Clinic of Dermatology, University of Catania, Catania, Italy; ⁷ Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy; ⁸ Unit of Clinical and Experimental Immunology, IRCCS Humanitas Clinic, Rozzano, Milan, Italy; ⁹ Department of Medical Biotechnologies and Translational Medicine, University of Milan, Milan, Italy; ¹⁰ IRCCS Fondazione Don Carlo Gnocchi, Milan, Italy; ¹¹ Department of Dermatology, University of Brescia, Brescia, Italy

Kaposi’s sarcoma (KS) is a lymphangioproliferative disorder associated with Human herpesvirus 8 (HHV8) infection. Four clinical subtypes are recognized: classic, endemic, epidemic (HIV-related) and iatrogenic. KS diagnosis is based on clinical features, histopathological assessment, and HHV8 serology. Classic KS is usually skin-limited and has a chronic course, while the iatrogenic variant may show mucosal, nodal or visceral involvement. Clinical staging is fundamental to guide the management. Localized disease may be treated with different local therapies, even if there are no randomized trials comparing these different modalities. Aggressive, disseminated KS and cases with visceral involvement usually require systemic chemotherapy, most commonly vinblastine, bleomycin or paclitaxel. Iatrogenic KS needs immunosuppression tapering/withdrawal and, if possible, switch to m-TOR inhibitors in post-transplant KS. The present work by a panel of Italian experts provides guidelines on KS diagnosis and management based on a critical review of the literature and a long and extensive personal experience.

KEY WORDS: Sarcoma, Kaposi; Herpesvirus 8, human; Diagnosis