Introduction: Kikuchi-Fujimoto disease or Kikuchi disease (KD) or histiocytic necrotizing lymphadenitis is an uncommon disease more frequent in Asia. It is diagnosed with lymph node histopathological findings.
Objective: To perform a systematic review of Kikuchi’s disease (KD) cases and describe clinical manifestations and associated etiology in the Tunisian population.
Methods: We conducted a multicentric descriptive study collecting patients with histologically proven KD. Clinical data, laboratory results, histopathologic examination, associated pathologies, and patient evolution were included.
Results: The search included 9 patients. They were 7 women and 2 males with a median age of 45 years old [24-72]. Common clinical manifestations were fever (n=8) and arthralgia (n=7). Lymph nodes mainly were bilateral (n=8), multiple (n=9), and in the cervical region (n=8) and axillary area (n=6). Laboratory results showed inflammatory syndrome and elevated lactate dehydrogenase. Areas of necrosis, karyorrhectic debris, and accumulation of histiocytes were specific histologic findings noted for all patients. Systemic Lupus Erythematosus was associated with KD in 2 patients and with Sjögren disease in one of them. Other associated pathologies were Lymphohistiocytic activation syndrome, lymphocytic meningitis, sepsis from a urinary tract infection, and idiopathic interstitial lung disease. Corticosteroids were prescribed in 4 cases. The other 5 cases had spontaneous regression of symptoms. The outcomes were favorable with recovery in 3 cases. Five patients developed Hodgkin lymphoma, myelodysplastic syndrome, and lupus nephropathy after KD diagnosis.
Conclusions: KD is exceptional in North Africa. The study noted the same clinical and histological findings in the literature. SLE was the most associated pathology. No ethnic variability was detected.
Sarcoidosis is characterized by the presence of noncaseating granulomatous inflammation in the affected organs. Isolated involvement of the hypothalamic-pituitary axis in patients with sarcoidosis is rare. We report a rare case of a female patient in whom hypophysitis, mimicking a pituitary macroadenoma, resulted in pituitary transsphenoidal surgery. A female patient had been complaining of bilateral temporal headaches for over a month. Brain MRI showed a pituitary adenoma of height 16 mm, width 16 mm, and depth 12 mm. Hormonal assay showed central hypothyroidism and elevated level of prolactin. Histological examination revealed granulomatous hypophysitis. A specific search for Mycobacterium tuberculosis was negative on the pituitary tissue. After the exclusion of differential diagnoses, the combination of clinical, laboratory and radiological tests led to the diagnosis of neurosarcoidosis. This report presents an uncommon case of a pituitary localization of neurosarcoidosis mimicking a macroadenoma. Understanding the different MRI aspects of neurosarcoidosis is essential to avoid interpretive blunders that could result in an incorrect diagnosis.
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