Kikuchi-Fujimoto Disease Associated to Connectivitis -About Two Cases-

Introduction: Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotizing lymphadenitis is a rare clinico-pathological entity of unknown etiology that primarily affects young Asian women. The association of this disease with dys-immune systemic disease is rare. We report two cases.

Observation 1: 28 year old woman, having systemic lupus erythematosus (SLE) with cutaneous, hematologic, and renal complication, who developed six years after cervical and axillary febrile lymphadenopathy. A lymph node biopsy showed histological and Immunohistochemical aspects suggesting KFD.

Observation 2: 30 year old woman, without medical history was hospitalized for febrile superficial poly -lymphadenopathy. Etiological investigation was negative. Lymph node biopsy confirmed the diagnosis of KFD. Two years later primary Sjogren’s syndrome was diagnosed in this patient.

Favorable evolution was noted in both cases after 1-2 months.

Conclusion: KFD is a very rare histological diagnosis. Underlying autoimmune conditions are rarely reported; particularly the association with primary Sjogren’s syndrome is exceptional. These associations represent a real diagnostic challenge and they deserve to be known by physicians.

Keywords: Kikuchi–Fujimoto’s disease; histiocytic necrotizing lymphadenitis; systemic lupus erythematosus ; primary Gougerot-Sjögren syndrome.