Hereditary angioneurotic edema is due to deficiency of
**Core Concept**
Hereditary angioneurotic edema (HANE) is a rare genetic disorder characterized by recurrent episodes of severe edema, primarily affecting the skin and mucous membranes. It is caused by a deficiency of a crucial protein involved in the regulation of the complement system, leading to an overactive inflammatory response.
**Why the Correct Answer is Right**
The correct answer is due to a deficiency of C1 esterase inhibitor (C1-INH), a protein that regulates the complement system and prevents the overactivation of the classical pathway. C1-INH is a serine protease inhibitor that controls the activity of C1r and C1s, two enzymes that initiate the classical pathway of complement activation. In HANE, the deficiency of C1-INH leads to an uncontrolled activation of the classical pathway, resulting in the release of various chemical mediators, including bradykinin, which causes the characteristic edema.
**Why Each Wrong Option is Incorrect**
* **Option A:** C4 is a component of the complement system, but its deficiency is associated with a different condition known as C4 deficiency, which presents with recurrent infections and immune complex-mediated diseases.
* **Option B:** Factor XII is involved in the intrinsic coagulation pathway and is not directly related to the pathogenesis of HANE.
* **Option D:** Prekallikrein is involved in the generation of bradykinin, but its deficiency is not the primary cause of HANE.
**Clinical Pearl / High-Yield Fact**
HANE is often misdiagnosed as an allergic reaction due to its association with urticaria and angioedema, but it is actually an autoimmune disorder caused by the deficiency of C1-INH.
**Correct Answer:** C. C1 esterase inhibitor.