Hepatocellular Focal Nodular Hyperplasia after Danazol Treatment for Hereditary Angio-oedema

Hepatocellular focal nodular hyperplasia after danazol treatment for hereditary angio-oedema.

Hereditary angio-oedema (HAE) is a potentially life-threatening disease manifesting as recurrent attacks of laryngeal, intra-abdominal, facial or peripheral oedema. The disease is caused by mutations in the gene coding for C1-inhibitor, resulting in low antigenic or functional concentrations of this inhibitor of complement and kinin systems. Long-term prophylactic treatment is indicated in pati...

Hepatocellular carcinoma after danazol treatment for hereditary angio-oedema.

Hereditary angio-oedema is characterised by recurrent episodes of laryngeal, intra-abdominal, facial or peripheral oedema. Danazol can be used as prophylaxis for recurrent attacks. Hepatotoxicity is a recognised adverse effect of danazol. We report an exceptional case of a danazol-induced hepatocellular carcinoma in a 75-year-old patient with hereditary angio-oedema.

Successful use of danazol for hereditary angio-oedema.

A 10-year-old boy with severe hereditary angio-oedema was given danazol, 200 mg each day. There was a pronounced reduction in the frequency and severity of oedema.

Management of hereditary angio-oedema with low-dose danazol.

Hereditary angio-oedema

Hereditary angio-oedema is caused by a heterozygous deficiency of C1 inhibitor. This inhibitor regulates several inflammatory pathways, and patients with hereditary angio-oedema have intermittent cutaneous or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days. In addition to orofacial angio-oed...