Zarrouk, S.1, El Euch, M.1,2*, Kefi, A.1, Ben Hamida, F.1,2, Ben Abdelghani, K.1, Turki, S.1 & Ben Abdallah, T.1
1Department of Internal Medicine “A”, Charles Nicolle Hospital, Tunis, Tunisia
2Research Laboratory of Kidney Diseases (LR00SP01), Charles Nicolle Hospital, Tunis, Tunisia
*Correspondence to: Dr. Mounira El Euch, Department of Internal Medicine “A” & Research Laboratory of Kidney Diseases (LR00SP01), Charles Nicolle Hospital, Tunis, Tunisia.
Copyright © 2020 Dr. Mounira El Euch, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Kikuchi-Fujimoto disease (KFD) is a subacute necrotizing regional lymphadenopathy. It’s a rare, benign and self-limited disease. We report a rare observation of a woman admitted with joint pain, which was related finally to KFD.
Kikuchi-Fujimoto disease (KFD) known as histiocytic necrotizing lymphadenitis is a subacute necrotizing regional lymphadenopathy. It’s a rare, benign and self-limited disease first described in Asia by Kikuchi and Fujimoto in 1972. Despite many studies in the literature, the etiology and pathogenesis of KFD remain unknown.
This patient was a 28-year-old female, without a past medical history that presented in May 2019 with a complaint of polyarthritis of large and small joints and an enlarged lymph node of the neck and the axillary region, fever 39°C - 40°C, nocturnal sweats, and an itchy macular rash of all 4 limbs, and a deterioration of general condition (weight loss of 5kg in 2 months), and a photosensitivity. The clinical examination showed hepatosplenomegaly. The biological analysis showed a normochromic normocytic anemia with a positive direct combs test IgG, and a leucopenia, positive CRP = 37.8mg/l. Radiological assessment showed a left axillary calcification, enlargement with irregularities of the sacral borders of the right sacroiliac joint and a triangular condensation of the foot of the left sacroiliac joint. The ophthalmological examination was normal. The immunological assessment showed positive anti-nuclear antibodies and positive anti RNP immunodot. B2 microglobulin value was 2.88 and angiotensin converting enzyme=67.46 (normal). The Echocardiography was normal. The cervical ultrasound showed multiple cervical ganglia of the right chain 1, 2, 3, 5 and right intra parotid. The thoraco-abdomino-pelvic scanner showed bilateral cervical and axillary lymphadenopathy, as well as homogeneous hepatomegaly. Lymph node biopsy with histological analysis showed necrotizing lymphadenitis of kikuchi. The evolution was spontaneously favourable.
Kikuchi-Fujimoto disease (KFD) is often associated with fever . Commonly reported in young adults of Asian ancestry, however a recent study in France showed worldwide cases . The cause of KFD still remains uncertain . From a histological point of view, KFD shows a para-cortical lymph node expansion with patchy along with well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils . Its diagnosis could be challenging, as lymphadenopathy and fevers are common symptoms of some more worrying diseases such as lymphoma and systemic lupus erythematosus . Clinical and histopathological correlation could help in the diagnosis of KFD, to avoid the use of unnecessary diagnostic procedures and inappropriate treatments . Clinicians and pathologists are poorly familiar with this entity, which could increase the diagnostic period and expenses .
Kikuchi-Fujimoto Disease is a rare disease which the precise physiopathology still remains unknown although postviral etiology or associated with autoimmune diseases such as SLD are currently being studied. Its classical manifestation is lymphadenopathy and fevers. Treatment is symptomatic, but if severe, corticosteroids may be considered. Even though it’s a benign disease and symptoms usually resolve within 6 months, there are reports of KFD being associated with poor outcomes. The purpose of this case report is to highlight the possibility of KFD in the differential of a neck mass or cervical lymphadenopathy. It also aims to popularize this disease in Tunisia.