Abstract
Conflict of interest: None declared. Sir, We report a 65‐year‐old man with a 12‐year history of atypical pyoderma gangrenosum (PG). Within the last 1 year his PG has shown a remarkable response to anticoagulation therapy. This appears to be related to an underlying dysfibrinogenaemia (qualitative fibrinogen defect) and is an association which has not previously been reported. This article further documents our patient's progress since his case was originally published because of severe intraoral involvement.1 Our patient first presented (aged 54 years) in 1993 with PG of his lower right leg. There was no evidence of an underlying disorder and treatment with prednisolone 25–40 mg daily had limited effect. Azathioprine and ciclosporin were poorly tolerated. By 1996, an IgA λ paraproteinaemia (6·0 g L−1) was detectable, with 3% plasma cells present in a bone marrow aspirate. No other abnormalities were identified and therapy with colchicine 500 µg twice daily resulted in temporary remission.1
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