Kikuchi-Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report.

BACKGROUND: Kikuchi-Fujimoto disease, which was originally described in young women, is a benign condition characterized by necrotizing lymphadenitis and fever. Even though the clinical course is usually self-limiting, it can be associated with recurrences and rarely can be associated with systemic lupus erythematosus or can be complicated with hemophagocytic lymphohistiocytosis. We report the case of a 17-year-old Sri Lankan Sinhalese schoolboy who presented with fever and cervical lymphadenopathy diagnosed as Kikuchi-Fujimoto disease and was complicated with hemophagocytic lymphohistiocytosis subsequently. Later he fulfilled the criteria for systemic lupus erythematosus. CASE PRESENTATION: A 17-year-old previously healthy Sinhalese schoolboy presented with high-grade fever associated with chills and rigors associated with loss of appetite and loss of weight for more than 40 days. On examination, he had bilateral firm matted tender cervical lymphadenopathy and firm hepatomegaly. An excision biopsy of his right cervical lymph node revealed necrotizing lymphadenitis and immunohistochemistry of a lymph node biopsy favored Kikuchi disease. Initial antinuclear antibody and anti-double-stranded deoxyribonucleic acid tests were negative and his C3 and C4 levels were normal. An infections screening was negative. He was treated with steroids. While in hospital he developed hemophagocytic lymphohistiocytosis and renal impairment. Later his antinuclear antibody titer became positive in 1:160 and fulfilled the diagnostic criteria for systemic lupus erythematosus. He was managed with steroids and immune suppressive drugs and showed remarkable improvement. CONCLUSION: Although Kikuchi-Fujimoto disease is uncommon in male patients, it needs to be considered in patients with lymphadenopathy and fever. The disease can be complicated with hemophagocytic lymphohistiocytosis and the patients need continuous monitoring for the possible development of systemic lupus erythematosus later in the course.

A Rare Cause of Ascites-Disseminated TB with Peritonitis in a Middle-Aged Female.

BACKGROUND: Disseminated tuberculosis (TB) has been increasingly recognized in adults in the recent times due to increased prevalence of immune suppression. Here we describe a case of 47-year-old female who presented with disproportionate ascites where the diagnosis of disseminated TB was delayed. CASE REPORT: A 47-year-old previously healthy female presented with generalised body swelling with disproportionate ascites and loss of appetite and weight for four-month duration. She denied any contact or past history of TB and reported no respiratory symptoms. Physical examination revealed significant ascites. There was no lymphadenopathy or hepatosplenomegaly. Respiratory system examination was normal. Her Erythrocyte Sedimentation Rate (ESR) was above 100. Tuberculin skin test was positive with 17mm. Contrast Enhanced Computed Tomography (CECT) abdomen revealed chronic liver disease with ascites. Diagnostic laparoscopy was in favour of miliary TB and the peritoneal biopsy revealed granulomatous inflammation with caseous necrosis, suggestive of TB. The patient was started on antituberculosis treatment and subsequently improved. CONCLUSION: TB peritonitis due to disseminated TB should be considered in the differential diagnosis of disproportionate ascites. Even though the diagnosis is difficult, diagnostic laparoscopy and biopsy is very helpful. It is important to have an early diagnosis since delay in treatment can be detrimental in most cases.