Co-occurrence of Kikuchi-Fujimoto’s disease and Still’s disease: case report and review of previously reported cases

Abstract

Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement. Given that the patient also met criteria for AOSD, a final diagnosis of KFD/AOSD co-occurrence was made. Anti-IL-1β therapy with anakinra resulted in rapid resolution of all symptoms. A literature search identified eight more cases of KFD/AOSD. Fever, rash, arthritis, and lymphadenopathy were present in all patients. No case report demonstrated an association of rash eruption clearly associated with fever spikes. Duration of symptoms ranged from 3 weeks to 10 years. Seven patients had leukocytosis, six had anemia, and five demonstrated elevated ferritin and/or decreased glycosylated ferritin. Seven patients had elevated erythrocyte sedimentation rate (ESR), and seven had transaminitis. Eight of nine patients had no evidence of infectious disease. Autoantibodies were absent from all patients. KFD and AOSD are very rare diseases, yet they may overlap. The two conditions not only share several clinical and laboratory characteristics but also differ in characteristic ways. Given the rapid response observed with anakinra in the index patient, IL-1β likely plays a role in both diseases.

Adult Onset Still's Disease With Multiple Lymphadenopathy: a Case Report and Literature Review

“…The xanthoma stage contains a large number of foamy histiocytes and few immunoblasts. When AOSD lymphadenopathy appears in a necrotic pattern, it needs to be differentiated from Kikuchi's disease, and the simultaneous appearance of both lesions has also been documented [12].…”

Kikuchi’s disease in a young Scandinavian woman

“…There are reports of co-occurrence of Kikuchi's disease and other diseases, such as Still's disease, where a higher proportion of patients have rashes and arthritis [7] and thyroid cancer, where Kikuchi's disease mimicked a metastasis [1].…”

“…Skin lesions may indicate an evolution into SLE. An association with other connective tissue diseases such as Still's disease, Hashimoto's thyroiditis, polymyositis, autoimmune hepatitis, Sjögren's syndrome, cutaneous necrotizing vasculitis, hemophagocytic lymphohistiocytosis, antiphospholipid syndrome, as well as mixed connective tissue disease is also suspected [5,7,10].…”

“…The unspeci ic symptoms and the rarity of the illness means that there are many other differential diagnoses to consider, e.g. lymphoma, tuberculosis, metastatic disease, infectious mononucleosis, systemic lupus erythematous (SLE) or cat scratch disease [1,[5][6][7][8].…”

Ritka betegség, atípusos bőrtünetekkel: felnőttkori Still-betegség

“…Differenciál-diagnosztikai szempontból infekciókat, malignus tumorokat, egyéb autoimmun betegségeket kell elsősorban kizárnunk, köztük a hasonló lázmenettel, rekurráló urticariform bőrtünetekkel, ízületi érintettséggel járó, de mindig monoklonális IgM gammopátiához kapcsolódó Schnitzlerszindrómát, illetve a hasonló labor eltérésekkel, nyaki limfadenopátiával, lázzal, hepatomegáliával járó, és a felnőttkori Still-betegséggel néhány esetben együtt jelentkező Kikuchi-betegséget (nekrotizáló hisztiocitás limfadenitisz) [3,8,20] .…”