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excluded in our patient.‘mount-Fuji Sign’ is a classic clue for the diagnosis of the

neurosurgical emergency - the tension pneumocephalus. hyperostosis frontalis interna which is a benign condition of no clinical significance should be included as a differential diagnosis in mount Fuji appearance of brain imaging.

References1. Steven J. michel. The mount Fuji Sign. Radiology 2004;232:449-450.

2. Amit Agarwal, Brij raj Singh. mount Fuji sign with concavo-convex appearance of epidural haematoma in a patient with tension pneumocephalus. Radiology Case 2009;3:10-12.

3. Josef G. heckmann, Oliver Ganslandt. The mount Fuji Sign. N Engl J Med 2004;350:1881.

4. rosemary she, Juliana Szakacs. hyperostosis frontalis interna: case report and review of Literature. Annals of clinical & Laboratory Science 2004;34:206-208.

5. Smith S, hemphill rE. hyperostosis frontalis interna. J Neurol Neurosurg Psychiatry 1956;19:42-45.

1Assistant Professor, 2Professor and head, 3Junior resident, Department of medicine, PSGImS&r, Peelamedu coimbatore 641004, Tamil Nadureceived: 31.08.2009; revised: 22.12.2010; Accepted: 23.12.2010

Kikuchi-Fujimoto’s Disease : A Report of Five CasesV Mukta1, K Jayachandran2, S Hemapriya3

Abstract Kikuchi Fujimoto’s disease is a rare, self limiting disorder characterized by fever and cervical lymphadenitis. It is often an incidental finding in a patient suspected to have tuberculosis or lymphoproliferative disorder. We diagnosed this disease in a young 23 year old female who was suffering from prolonged fever, cervical lymphadenopathy, anemia, leucopenia and mild splenomegaly; based on histopathological study of the excised lymphnode. We reviewed the clinical records and histopathologic findings of four more patients of Kikuchi-Fujimoto’s disease, who were treated by others at our institute. All five patients improved with NSAIDS (non steroidal anti inflammatory drugs). histopathological finding of histiocytic necrotizing lymphadenitis or kikuchi’s disease proved invaluable in characterization of these cases.

Introduction

Kikuchi – Fujimoto’s disease is a self limited disease1, characterized by fever and cervical lymphadenitis .

Diagnosis is established by excision lymph node biopsy and histopathological study2,3. It is a benign disease of unknown etiology and symptoms resolve with NSAIDS. Nevertheless , it is a close differential diagnosis for tuberculosis and autoimmune disorders and is often wrongly treated with empirical antituberculous drugs. Follow up of these cases is a must, in order to detect any autoimmune connective tissue disorder which may develop at a later stage4,5 . We present here a report of five cases from a teaching hospital in Coimbatore, Tamilnadu.

Case ReportsCase 1

A 23 year old female from kerela presented with fever of 21/2 months duration. She complained of weight loss and poor appetite. She had been treated ouside with empirical antituberculous drugs for 1 month. On examination and investigations, she was found to have severe microcytic hypochromic anemia, leucopenia; mild splenomegaly and bilateral posterior cervical ,enlarged, nontender, nonmatted lymph nodes ; largest measuring 2x2cm in size. ANA and hIV serology was negative . Excision biopsy of the lymph node was suggestive of kikuchi’s disease. She improved with nonsteroidal anti-inflammatory drugs (NSAIDS). Blood transfusion and hematinics were given.Case 2

A 20 year old female from coimbatore presented with fever for 2 days and neck mass on the right side. Past history of pulmonary tuberculosis was present. Examination and

investigations revealed right sided cervical lymphadenopathy 4x3 cm in size, nontender , nonmatted. Mantoux was negative. ANA , dsDNA and hIV serology was negative . Excision biopsy of lymphnode was suggestive of kikuchi’s disease. She improved with NSAIDS.Case 3

A 30 year old female from Tiruppur was admitted with history of fever of 1 month duration. Examination and investigations revealed enlarged right sided deep cervical lymph node , splenomegaly and microcytic hypochromic anemia. mantoux was 10 mm in size. hIV serology was negative . Excision biopsy of lymph node was suggestive of kikuchi’s disease. She was treated with empirical antituberculous drugs and NSAIDS . Patient was lost to follow up.Case 4

A 23 year old male from Erode was admitted with high grade fever for 10 days. Examination and investigations revealed enlarged right upper deep cervical and posterior auricular lymph nodes , elevated ESr, LDh , liver enzymes; ANA serology was not done. hIV serology was negative. Excision biopsy of lymph node was suggestive of kikuchi’s disease. he was started on empirical antituberculous drugs and NSAIDS. Antituberculous drugs were stopped once biopsy report was obtained. Patient was asymptomatic on follow up.Case 5

A 20 year old male presented with fever for 10 days and left sided neck swelling of 2 weeks duration. Examination and investigations revealed 3x3 cm posterior cervical lymphadenopathy. ANA serology was not done. hIV serology was negative . Excision biopsy of lymph node was suggestive of kikuchi’s disease . he improved with NSAIDS.

Lymph node biopsy in all the five cases, showed areas of necrosis, karryorhexis, histiocytes , plasmacytoid monocytes with complete absence of neutrophils and granulomas on histopathological examination. The lymphnode specimen stained

184 © JAPI • mArch 2011 • VOL. 59

negative for AFB. (Figures 1, 2- representative photomicrographs of case1)

DiscussionKikuchi Fujimoto’s disease is self limiting in nature.

Symptoms resolve with NSAIDs. Diagnosis is based on histopathological examination of the excised lymphnode. Kikuchi’s disease is characterized by paracortical necrotizing lesions, abundant karrhyorrhectic debri, large mononuclear cells; scanty plasma cells and neutrophils.2

Our patients included 3 females and 2 males who had fever and lymphadenitis. Two had varying degrees of anemia ; leucopenia was seen in 2 patients and two had splenomegaly. Tuberculosis, lymphoma and autoimmune disorder were close differentials. Excision biopsy was diagnostic, the histopathological picture consisted of areas of necrosis, karryorhexis, histiocytes, plasmacytoid monocytes with complete absence of neutrophils and granulomas. The lymphnode specimen stained negative for AFB. Lymphadenopathy regressed with NSAIDS. Patients were asymptomatic at discharge.

Fig. 1 : Low power view showing extensive necrosis and lack of granulomas

Fig. 2 : Oil immersion view showing histiocytes and plasmacytoid monocytes

clinicians should be aware of the benign nature of kikuchi’s disease and avoid unwarranted antituberculous therapy. connective tissue disorders should be excluded in all cases. Follow up is needed for several years. It should be considered in the differential diagnosis of all cases of fever with lymphadenopathy.

References1. Nieman rB. Diagnosis of Kikuchi’s disease. Lancet 1990;335:295.2. Turner rr, martin J, Dorfman rF. Necrotising lymphadenitis, a

study of 30 cases. Am J Surg Pathol 1983;7:115-23. 3. menasce LP, Banerjee SS, Edmondson D, harris m. histiocytic

necrotising lymphadenitis (Kikuchi-Fujimoto disease): continuing diagnostic difficulties. Histopathol 1998;33:248-54.

4. Santana A, Lessa B, Galrao L, Lima I, Santiago m. Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 2005;24:60-3.

5. chen Yh, Lan JL. Kikuchi disease in systemic lupus erythematosus: clinical features and literature review. J Microbiol Immunol Infect 1998;31:187-92.

*Professor, **resident, ***consultant cardiologist, Department of medicine, Institute of medical Sciences, BhU and rajapuria heart Hospital, Varanasi 221 005, Uttar Pradeshreceived: 09.10.2009; Accepted: 31.12.2009

Rupture of Non-coronary Sinus of Valsalva with Infective Endocarditis of Naive Tricuspid Valve Presenting with Recurrent Ill-sustained Ventricular TachycardiaNK Singh*, M Godara**, V Agrawal***

AbstractInfective endocarditis affecting the tricupid valve due to rupture of non-coronary sinus of Valsalva is distinctly rare. We are reporting such a rare case, presenting with recurrent ill sustained ventricular tachycardia.

Introduction

Even in the modern era of advanced diagnostic imaging and potent antimicrobial therapy, infective endocarditis (IE)

continues to surprise, frustrate and perplex, and remains an evolving disease with persistenty high mortality and morbidity.1,2