Clinical, Cosmetic and Investigational Dermatology (Aug 2021)
Cutaneous Macroglobulinosis Presenting as Serpiginous Purpura, a Case Report and Literature Review
Abstract
Siriorn Sukanjanapong,1 Kumutnart Chanprapaph,1 Suthinee Rutnin,1 Paisan Boonsakan,2 Sulada Pukiat3 1Division of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 3Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Kumutnart ChanprapaphDivision of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, ThailandTel +66-2-2011141Fax +66-2-2011211Email [email protected]: Cutaneous macroglobulinosis is a rare manifestation of Waldenstrom macroglobulinemia when there is deposition of IgM in the dermis. The clinical presentation varies from skin colored to pink papules and ulcerative nodules on trunk, extensor surfaces of upper and lower limbs to hyperkeratotic lesions on the soles. We herein report a 78-year-old-male with Waldenstrom macroglobulinemia who presented with serpiginous purpura. The differential diagnoses included occlusion of vessel lumen by the lymphoplasmacytoid cells, high level of IgM or cryoglobulin, as well as small to medium vasculitis secondary to Waldenstrom macroglobulinemia. The histopathology revealed vasculopathy and vasculitis, while further immunohistochemistry highlighted deposition in the vessel lumen and vessel wall with IgM, suggesting the diagnosis of cutaneous macroglobulinosis. In this case report, we discuss this rare presentation and reviewed previous cases of cutaneous macroglobulinosis.Keywords: Waldenstrom macroglobulinemia, lymphoplasmacytic lymphoma, monoclonal gammopathy, vasculitis, vasculopathy
