Cutaneous Involvement in Patients With Angioimmunoblastic Lymphadenopathy With DysproteinemiaA Clinical, Immunohistological, and Molecular Analysis

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Abstract
ANGIOIMMUNOBLASTIC lymphadenopathy with dysproteinemia (AILD) is a clinicopathologic syndrome characterized by fever, night sweats, weight loss, generalized lymphadenopathy, hepatomegaly, and splenomegaly.1-5 Histological examination of lymph nodes typically shows an effacement of lymph node architecture, a polymorphous infiltrate including immunoblasts, small lymphocytes, plasma cells, eosinophils, epithelioid histiocytes, and a prominent arborizing postcapillary vasculature. Hematologic findings usually show anemia, leukocytosis, and polyclonal hypergammaglobulinemia.5 The overall clinical course of AILD is aggressive, with a mortality rate ranging from 50% to 72% and a median survival from 11 to 30 months.6,7 The major causes of mortality are infection and transformation to an aggressive lymphoma.4,8-12 The disease is considered to be a subtype of peripheral T-cell lymphoma. However, the pathogenesis of the disease remains unclear; some authors consider AILD to be a primary monoclonal T-cell disorder, whereas others suggest a primary polyclonal proliferation, sometimes triggered by drug administration or viral infection, leading to a secondary monoclonal T-cell proliferation.13