Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Response of variant hereditary angioedema phenotypes to danazol therapy. Genetic implications.

Hereditary angioedema (HAE), an auto-somal dominant disorder characterized by attacks of episodic edema is associated with decreased functional levels of the C1 esterase inhibitor. Approximately 85% of patients have lowered antigen levels of a normal inhibitor protein. 15% of patients have normal or elevated antigenic levels of functionless protein. We have examined the response to danazol ther...

Refractory Hailey-Hailey Disease That Responded Well to Photodynamic Therapy.

of vascular origin. The most important differential diagnoses are angiosarcoma, which expresses endothelial markers such as CD31 and CD34, and chronic expansive hematoma, which presents as an encapsulated tumor filled with blood and neovasculature. What was interesting about this case, apart from the rarity of the condition, was the ease with which a giant vascular eccrine spiradenoma could be ...

Acquired angioedema

Acquired angioedema (AAE) is characterized by acquired deficiency of C1 inhibitor (C1-INH), hyperactivation of the classical pathway of human complement and angioedema symptoms mediated by bradykinin released by inappropriate activation of the contact-kinin system. Angioedema recurs at unpredictable intervals, lasts from two to five days and presents with edema of the skin (face, limbs, genital...

Acquired angioedema responding to rituximab.

Long-term efficacy of danazol treatment in hereditary angioedema.

BACKGROUND No systematic study has been published yet on the long-term efficacy of attenuated androgens in hereditary angioedema (HAE). Our aim was to conduct a follow-up study in two (German and Hungarian) cohorts of HAE patients (45 and 39 patients, respectively) undergoing uninterrupted treatment for 6 years with similar (starting dose 128 ± 78 mg per day and 136 ± 70 mg per day, respectivel...