Indian doctors report an unusual cause of fever: Behcet syndrome

An unusual cause of fever has been published in the Journal of the Association of Physicians of India. Dr Thenmozhi Damodaran at Dr Rangarajan Memorial Hospital, Chennai, Tamil Nadu and associates have reported a case of Behcet syndrome presenting as high-grade fever with orogenital ulcers.

Behcet syndrome is an autoimmune multisystem disorder often affecting young adults and its pathological origin is unclear. Behçet syndrome was first described by Hippocrates in the 5th century. The syndrome carries the name of the Turkish dermatologist Hulusi Behçet, who, in 1937, described a syndrome of recurrent aphthous ulcers, genital ulcerations, and uveitis leading to blindness.

According to history a 24-year-old male presented with complaints of high-grade intermittent fever for 45 days duration. He also gives a history of the development of painful ulcers in the groin region which initially started as a papule and later burst open to form an ulcer. He also had multiple painful oral ulcers for 10 days duration. He reported having a significant weight loss of ~ 15 kg in one month. He was treated outside with multiple antibiotics for fever. There was also a history of similar illness 1year back which resolved with treatment and treatment details were not available.

Th examination of the oral cavity revealed multiple aphthous ulcers. He also had three well-circumscribed ulcers, one in the root of the right scrotum and other two similar ulcers in left inner thigh with minimal serous discharge without lymphadenopathy.

Routine blood investigations revealed elevated total counts of 16,530 cells/cu.mm with neutrophilic predominance, Hb-19.9g/dl, PCV-53.7%, RBC count-6.14million cells/cu.mm and platelet counts of 3.71lakhs/cu.mm. Repeat haemoglobin showed a decreasing trend. Renal parameters were normal. Blood and urine cultures were sterile. Routine fever work up such as MPQBC, typhoid IgM, Scrub typhus IgM, Leptospira IgM were negative.

Pathergy test was done which was positive –pustules were formed at the site of needle prick (PANEL C). HLA B51 was positive which confirmed the diagnosis of BEHCET syndrome. Consultant physician of infectious diseases opinion was obtained and concurred with the treatment.

He was treated with IV fluids, antipyretic, oral antibiotics and later started on oral colchicine. Regular follow up showed resolving oral and genital ulcers and the patient remained afebrile.

Two months later, he presented with acute onset of a headache and blurring of vision in the right eye for a one-week duration. On examination of the central nervous system revealed right lateral rectus palsy with bilateral papilledema. Perimetry showed visual field defects in the right eye. MRI brain with MRV showed thrombosis of superior sagittal sinus and medial aspect of transverse sinuses with bilateral papilledema. He was then initiated on oral anticoagulation and therapeutic INR was maintained. Consultant Rheumatologist concurred with the same line of management. He showed gradual improvement in his neurological signs in subsequent follow-ups.

He later developed swelling of left lower limb which he left untreated. He was non-complaint with oral anticoagulation therapy. He presented with complaints of painful swelling of the right lower limb. Doppler study of the venous system of both the lower limbs showed acute DVT of the right lower limb and chronic DVT of the left lower limb. His haematological evaluation showed persistently elevated haemoglobin levels with increased PCV for which consultant Haematologist opinion was obtained.

He advised venesection for persistent polycythaemia (Likely secondary polycythaemia). Coagulation profile was normal. His thrombophilia workup was negative for antiphospholipid and anticardiolipin antibodies with increased serum homocysteine levels (21.29micromol/L) while evaluation of other thrombophilia parameters were planned later. He was restarted on oral anticoagulation therapy along with B complex and folic acid therapy.

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