Acquired C1-inhibitor deficiency: a case report

SUMMARY

Angioedema due to C1 – inhibitor deficiency may be hereditary (HAE) or acquired (AAE). AAE is a very rare condition, whose prevalence is possibly underestimated, as it is often unrecognized. AAE usually occurs after the fourth decade of life, and it is commonly associated to an underlying disease, mainly lymphoproliferative disorders. We report a 74-years old woman with recurrent episodes of angioedema involving upper airways in which diagnosis was obtained a long time after symptoms onset. A concomitant B cell leukaemia was also diagnosed. This report stresses the importance of the recognition of AAE: a delayed diagnosis is indeed associated to the risk of severe lifethreatening episodes, unresponsive to the usual therapy of common form of angioedema. AAE should be considered as a possible diagnosis in subjects with adult onset of angioedema: a careful clinical history is basic, since the clinical features and the timecourse are often suggestive of the disease.