9. Hypomyopathic dermatomyositis complicated by mannose-binding lectin deficiency

摘要

IntroductionConnective tissue diseases can present a diagnostic odyssey both for the patient and the practitioner. Here, the clinical journey of a patient with hypomyopathic dermatomyositis and mannose-binding lectin deficiency is described, demonstrating the interaction between complex co-morbidities and highlighting the importance of recognising different phenotypes of dermatomyositis. The case addresses the therapeutic challenges of conflicting immunological requirements, the importance of monitoring for infective complications, and is also a rare example of long-term IV immunoglobulin therapy.