Systemic Lupus Erythematosus Lymphadenopathy Presenting as Kikuchi-Fujimoto Disease in an Adolescent

Abstract

Systemic lupus erythematosus is a multisystem autoimmune disorder with a highly heterogeneous clinical presentation. The clinical phenotype varies from mild cutaneous and musculoskeletal manifestations to neurological involvement. Lymphadenopathy is a frequent manifestation of SLE, but the association is often not recognized, as lymphadenopathy is not a criterion for diagnosis. An unusual and seldom reported mimicker of lupus lymphadenitis is Kikuchi-Fujimoto Disease. This is a rare self-limiting disease of young adult females that presents with lymphadenopathy, fever, and systemic symptoms. Lupus lymphadenitis and KFD share some common clinical and pathologic features; but distinguishing between those two diseases can be challenging. We describe a 16-year-old Hispanic female who presented with axillary lymphadenopathy and was initially diagnosed with KFD based on an excisional lymph node biopsy; but later met the criteria for the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria SLE. This case highlights the need for clinicians to be aware that patients with SLE may present with lymphadenopathy and to consider the association between Kikuchi disease and SLE to prevent misdiagnosis and allow for timely treatment to avoid complications.

Keywords: kikuchi; kikuchi-fujimoto; lupus lymphadenitis; lymphadenitis; necrotizing lymphadenitis; systemic lupus erythematosus.

Figure 1. Hematoxylin-eosin-stained section (10X) show axillary lymph node with architecture distorted by pale areas composed of eosinophilic, granular material, karyorrhectic debris and interspersed lymphocytes and histiocytes. No hematoxylin bodies or aggregates of large atypical lymphocytes are identified.

Figure 2. Hematoxylin-eosin-stained section (400x) of the axillary lymph node show lymphocytes with areas of necrosis, abundant karyorrhectic debris, and numerous histiocytes.