Unusual Coincidence of the Hereditary Angioedema, Systemic Lupus Erythematodes and Tongue Carcinoma


Authors: P. Králíčková ;  E. Burešová ;  J. Krejsek ;  I. Krčmová
Authors‘ workplace: Ústav klinické imunologie a alergologie LF UK a FN, Hradec Králové, přednosta prof. RNDr. J. Krejsek, CSc.
Published in: Otorinolaryng. a Foniat. /Prague/, 60, 2011, No. 3, pp. 163-168.
Category: Case History

Overview

Hereditary angioedema (HEA) caused by C1 esterase inhibitor deficiency is a rare autosomal dominant inherited disorder. It is characterized by recurrent episodes of potentially life-threatening swellings without itching localized in the dermis and submucosa. The most dangerous is laryngeal edema. We report a case of the young woman in whom the correct diagnosis of HEA was delayed 11 years. Hereditary angioedema in this patient was presented not only as episodes of localized head and extremities skin swellings but also as painful gastrointestinal attacks. She revealed several severe nasopharyngeal and laryngeal attacks. Systemic lupus erythematodes and tongue carcinoma as independent complications were evolved in time. This report underlines the fact that hereditary angioedema is often neglected in differential diagnoses. If hereditary angioedema is diagnosed, effective treatment which might be life-saving and highly improving patients’ quality of life is available. .

Key words:
angioedema, C1 inhibitor, icatibant, danazol, lupus, tongue carcinoma.


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Audiology Paediatric ENT ENT (Otorhinolaryngology)

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