Letters to the Editor
Indian Pediatrics 2003; 40:907-908
Antiphospholipid Antibody Syndrome as a Cause of Budd-Chiari Syndrome
We read with interest the case report
on cutaneous manifestations of the Antiphospho-lipid Antibody Syndrome (APS)
in a recent issue of this journal(l). Soon, thereafter, we diagnosed a
child with APS. A six-year-old male presented to us with intermittent
abdominal pain of three years duration and painful palms and soles.
Examination revealed tender hepatomegaly and tender palmar erythema. The
tips of the fingers had a bluish hue and the tip of the thumb showed a
fine line of demarcation almost suggestive of early gangrene. Liver
function tests were normal. Ultrasonography suggested Budd-Chiari
syndrome (BCS). This was confirmed on digital subtraction angiography,
which revealed narrowing of the hepatic veins and stenosis of the
suprahepatic inferior vena cava. He underwent stenting for the same. The
anticardiolipin IgM antibody test and lupus anticoagulant profiles were
unequivocally positive. This established a diagnosis of APS in keeping
with the recently published criteria(2). He is currently on warfarin
therapy and doing well 4 months after the diagnosis.
Venous thrombosis in various organs and tissues (especially occlusion of the deep veins of the lower limb) is the most frequently reported presentation in the pediatric age group. APS is now considered as one of the most frequent causes of BCS, though BCS itself may not be a very common presentation of APS(3). Hence it is important to include an antiphospholipid antibody profile in evaluation of all patients diagnosed with BCS.
Arterial thrombosis is however much more common in the younger Children, i.e., 62% of less than ten-year olds(3). Hence young children with stroke should be screened for antiphospholipid antibodies, where they account for upto 18% of the cases(4).
Besides the cutaneous manifestations listed by Bapat, et al.(1) rarer skin lesions include palmar-plantar erythema (as in our case), subungal splinter hemorrhages, erythe-matous macules and tender skin nodules (5). In a scenario with co-existing liver disease (BCS) and APS, palmar erythema may be mistaken as a sign of liver cell failure.
The clinical spectrum of APS in children is wide and the condition is probably not as rare as is thought. Colleagues are urged to consider this entity in the etiological diagnosis of every vascular occlusion syndrome they may encounter.