Post‐Angioedema Attack Skin Blisters: A Unique Presentation of Hereditary Angioedema

1Cleveland Clinic, Cleveland, OH
2Case Western Reserve University, Cleveland, OH
3Cleveland Clinic, Cleveland, OH
4Cleveland Clinic, Cleveland, OH

Meeting: Hospital Medicine 2010, April 8-11, Washington, D.C.

Abstract number: 381

Case Presentation:

A 36‐year‐old male with a known history of hereditary angioedema presented to an outside hospital ER with acute respiratory failure requiring endotracheal intubation. The patient received 2 units of fresh frozen plasma after intubalion and was subsequently transferred to our hospital. In his previous angioedema episodes, his hands, feel, and genilalia had been involved, and in 2002 he had an episode of small bowel edema; all episodes generally occurred after physical trauma. The patient had been prescribed prophylactic danazol 100 mg BID, but had gradually been reducing the dosage because of concerns of secondary hypogonadism; at the time of presentation he had tapered himself down to 100 mg danazol q M/W/F. He was extubated 3 days into his MICU course after resuming danazol as previously prescribed and receiving 6 additional units of FFP. Laboratory studies revealed C4 10 mg/dL and C1 esterase inhibitor 10 mg/dL (both were low). After resolution of the orofacial swelling, he developed bullous eruptions in the interdigital spaces of both hands that were noted 1 day after extubation. The vesicles were painful and clear. A dermatology consultation was sought, with subsequent biopsies and cultures negative for infectious etiologies. It was thought that the lesions represented acute edema blisters secondary to the angioedema episode. The lesions resolved spontaneously.

Discussion:

Hereditary angioedema is a well‐recognized entity affecting extremities, face, and intra‐abdominal organs, most often caused by mutations in the C1‐inhibitor gene with secondary bradykinin‐medialed increased vascular permeability. After an angioedema attach resolves, it is rare that new manifestations ensue. Our patient presented with new hand vesicles after the initial event resolved.

Conclusions:

The hospitalist should recognize that the development of edema blisters after resolution of a fare of hereditary angioedema (only 1 previous case report) is related to the primary event and hence avoid unnecessary dermatologic diagnostic workup and treatment.

Author Disclosure:

M. Auron, none; K. Schlosser, none; J. Wiesen, none; M. Naeem, none.

To cite this abstract:

Wiesen J, Schlosser K, Naeem M, Auron M. Post‐Angioedema Attack Skin Blisters: A Unique Presentation of Hereditary Angioedema. Abstract published at Hospital Medicine 2010, April 8-11, Washington, D.C. Abstract 381. Journal of Hospital Medicine. 2010; 5 (suppl 1). https://www.shmabstracts.com/abstract/postangioedema-attack-skin-blisters-a-unique-presentation-of-hereditary-angioedema/. Accessed August 19, 2019.

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