title: Recurrent histiocytic necrotizing lymphadenitis with a long latency in a patient with autoimmunity: a case report and review of literature.
abstract: Kikuchi-Fujimoto disease (KFD), a histiocytic necrotizing lymphadenitis (HNL), characteristically presents as cervical lymphadenopathy in young Asian women. Most resolve spontaneously with rare recurrences described. We report a patient with biopsy-proven recurrence of KFD-like HNL after almost 8 years and analyze 65 additional published cases with recurrences. While those with recurrences similarly affect young (average age = 27 years), Asian (80%) women (76%), 73% had multiple sites of involvement and 32% of those tested had underlying autoimmune conditions. Our case is unusual with respect to the following: (a) Age: 50 years, the oldest among the reported patients with recurrences. (b) Race: African descent, with only 3 others reported with recurrent HNL. Of these 4 cases, 2 had underlying autoimmunity. (c) Underlying condition: Her clinical and laboratory features were best felt to represent Sjögren's syndrome (SjS). Only 2 other cases of SjS-associated HNL have been reported; in 2 recently reported cases SjS developed subsequently.
authors.affiliation: Department of Pathology, Division of Hematopathology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA 02115, USA.
authors.forename: Agata M
authors.initials: AM
authors.lastname: Bogusz
authors.forename: Parul
authors.initials: P
authors.lastname: Bhargava
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D015897:Comorbidity; D005260:Female; D020042:Histiocytic Necrotizing Lymphadenitis / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D006801:Humans; D012008:Recurrence; D012859:Sjogren's Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D013997:Time Factors