Edema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor

TL;DR

A rare case of acquired angioedema with low C1-inhibitor presented as swelling in the scrotum and penis, highlighting the importance of accurate diagnosis.

Contribution

This case report highlights an uncommon initial presentation of AAE-C1-INH and emphasizes the need for proper diagnosis to avoid complications.

Findings

A 79-year-old patient presented with painless swelling in the scrotum, penile shaft, and extremities.

Lab values confirmed acquired angioedema without lymphoreticular or rheumatic disorders.

Proper diagnosis is crucial to prevent airway compromise and ensure appropriate treatment.

Abstract

Acquired angioedema with low C1-inhibitor (AAE-C1-INH) is a rare disorder characterized by an acquired deficiency in the C1 esterase inhibitor (C1-INH). This case report describes a 79-year-old patient presenting to the emergency department for painless swelling of his scrotum, penile shaft, and left lower and upper extremities with lab values consistent with acquired angioedema without identifiable lymphoreticular or rheumatic disorder on history, exam, or total body PET scan. Proper diagnosis of AAE-C1-INH is essential to prevent life-threatening airway compromise, ensure proper therapy, and exclude lymphoreticular disorders as the etiology of AAE-C1-INH.

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