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Having recently started work in the rheumatology department of the Norfolk and Norwich Hospital I read with great interest the article on Hug(h)e(s’) ears: an unusual presentation.1 Amazingly we have recently seen an almost identical, but less catastrophic case.
A 27 year old white man presented with a 24 hour history of ears so swollen and painful, that he could not lie in bed with them touching the pillow. One year previously he was diagnosed with primary antiphospholipid syndrome (APLS) after recurrent deep vein thromboses and a raised IgG anticardiolipin antibody at 92 iu/ml. He was subsequently given warfarin.
On admission this time, his INR, while taking warfarin 7 mg per day, was subtherapeutic at 1.6. Biopsy from his left ear lobe showed numerous fibrin thrombi with no associated vasculitis, consistent with thrombosis secondary to APLS.
His warfarin dose was increased to obtain an INR between 3 and 4. Within a few days he had recovered and was discharged home well.
Perhaps Hughes’ ears should be renamed Norfolk ears?
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Authors’ reply
We note with interest the report from Dr Gaffney. While the coincidence is indeed curious, these cases do suggest possible mechanisms for activation of thrombosis. The external ear is characterised particularly by a lower average temperature than core body temperature, and by its susceptibility to trauma and pressure effects. In our case, cryoglobulins were not identified, and no comment in this regard is made by the authors. There is no specific reference to any aural trauma, though presumably, as in our case, it is difficult to assess what pressure was exerted on the external ear during sleep. It is plausible that such pressure causes a degree of blood stasis, which together with inadequate anticoagulation, resulted in thrombosis. Such speculation may be interesting, but it is this latter point that deserves emphasis—patients with antiphospholipid syndrome who have had thrombi will do so again, potentially with serious consequences, if the INR is not scrupulously maintained above 3.0, a message that must be spread widely: “Friends, Norfolk countrymen, lend me your ears!”