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Indian Pediatr 2013;50: 711-712 |
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Behçet Disease Presenting as Deep Vein
Thrombosis and Epididymoorchitis
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Mohammed Ramzan and
Satya
Prakash Yadav
Pediatric Hematology Oncology and BMT Unit, Department
of Pediatrics, Sir Ganga Ram Hospital, Delhi, India.
Email: [email protected]
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Behçet’s Disease is a chronic, multisystemic, inflammatory
disorder characterized by intraocular inflammation, oral and
mucosal ulcerations, skin lesions, and a variety of other
manifestations [1]. It occurs rarely in children [2]. A
15-year-old boy was admitted with the complaints of pain in
right calf and right testis for 3 days. He had past history
of three episodes of high grade fever with nonspecific
muscular pain and testicular pain in last one year. He also
had a history of recurrent aphthous oral ulcerations that
occurred three-to-four times per year accompanied by high
fever. On examination, oral apthous ulcers were present, an
ulcer was present on right side of the scrotum and his right
testis and epidydimis were swollen and tender.
Further examination revealed old healed lesions of erythema
nodosum over left leg. His right calf muscle was swollen and
tender and Homan’s sign was positive. Eye examination
revealed anterior uveitis.
Hemoglobin, leukocyte count and platelet
count were 12.6 g/dL, 15,200/mm 3
and 3, 50,000/mm3,
respectively. C-reactive protein level and erythrocyte
sedimentation rate were 7.0 mg/dL and 93 mm/hour,
respectively. He had no history of sexual exposure and his
HIV and VDRL both were negative. Doppler ultrasound of the
right leg showed popliteal vein thrombosis. Subcutaneous low
molecular weight heparin 1 mg/kg/dose twice daily was
started. Pathergy test and HLAB51 were positive.
Investigations for thrombosis, serum protein C and S,
homocysteine, anti-nuclear antibody, anti-phospholipid
antibody, Lupus anticoagulant and Antithrombin III levels
were normal. Child was started on intravenous
methylprednisolone 2 mg/kg/day, and improved markedly. He
was discharged on oral Warfarin and oral prednisolone and
was in excellent condition at follow-up one month later.
As per International Study Group for
Behçet disease, the presence of oral ulcerations in addition
to the presence of two criteria from among recurrent genital
ulcerations, ocular lesions, skin lesions or positive
pathergy test is sufficient for diagnosis of Behcet disease
[3]. In our case, the presence of the oral and genital
ulcers and uveitis with a positive pathergy test confirmed
the diagnosis. The presence of both deep vein thrombosis and
epididymo-orchitis in a patient with Behcet disease has not
been described so far in children, though individually these
presentations are known [4,5].
References
1. Marshall SE. Behcet’s disease. Best
Pract Res Clin Rheumatol. 2004;18:291-311.
2. Kone-Paut I, Gorchakoff-Molinas A,
Weschler B, Touitou I. Paediatric Behcet’s disease in
France. Ann Rheum Dis. 2002;61:655-6.
3. International Study Group for Behcet’s
Disease. Criteria for diagnosis of Behcet’s disease. Lancet.
1990;335:1078-80.
4. Pektaº A, Devrim I, Besbas N, Bilginer
Y, Cengiz AB, Ozen S. A child with Behcet’s disease
presenting with a spectrum of inflammatory manifestations
including epididymoorchitis. Turk J Pediatr. 2008;50:78-80.
5. Pande I, Uppal SS, Kailash S, Kumar A, Malaviya AN.
Behcet’s disease in India: A clinical, immunological,
immunogenetic and outcome study. Br J Rheumatol.
1995;34:825-30.
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