Adult-Onset Still's Disease With Extensive Lymphadenopathy Mimicking Lymphoproliferative Malignancy

Abstract

Adult-onset Still's disease (AOSD), a rare systemic inflammatory disorder of unknown etiology, is considered in broad differential in patients with fever of unknown origin or unexplained lymphadenopathy. It is characterized by spiking fever, evanescent salmon-colored maculopapular rash, arthritis or arthralgia, and leukocytosis. Due to broad differentials and lack of any specific diagnostic tests, diagnosis of AOSD poses a great challenge. A concerned physician should have a high index of suspicion while dealing with patients presenting with clinical symptoms of this systemic disorder. We report a case of a 25-year-old African American female with the past medical history of AOSD, who presented with four weeks history of extensive cervical and axillary lymphadenopathy mimicking lymphoproliferative malignancy. Cases have been reported with the development of malignant lymphoma during the course of AOSD. Therefore, careful monitoring of patients with regular follow-up is vital as these patients may develop lymphoproliferative malignancy in the future.

Keywords: adult-onset still’s disease; lymphadenopathy; lymphoma; lymphoproliferative malignancy; polyarthritis.

Figure 1. CT scan of neck with IV contrast showing bilateral cervical lymphadenopathy with the largest one measuring approximately 2.2 x 3.5 cm lymph node in the right submandibular region

Figure 2. CT scan of chest with IV contrast showing bilateral axillary lymphadenopathy with the largest one measuring approximately 3.1 x 2.4 cm lymph node in the right axilla

Figure 3. Lymph node is enlarged with reserved architecture and follicular hyperplasia. H&E stain, 20x (a) and 40x (b)

Figure 4. (a) CD21 shows expanded follicular dendritic cell (FDC) meshwork, 20x and (b) BCL2 is negative in the germinal centers