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THROUGH THE LENS
Year : 2015  |  Volume : 6  |  Issue : 6  |  Page : 462-463  

Acute and dramatic saxophone penis


Department of Dermatology, Hospital Universitario 12 de Octubre, Madrid, Spain

Date of Web Publication17-Nov-2015

Correspondence Address:
Carlota Gutiérrez García-Rodrigo
Department of Dermatology, Hospital Universitario 12 de Octubre, Avenida de AndalucÍa s/n, 28041 Madrid
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.169726

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   Abstract 

We present a case of intense genital swelling because of a hereditary angioedema. This rare disease should be included in the differential diagnosis of acute and asymptomatic genital edema, because it may prevent future potentially life-threatening episodes of visceral angioedema.

Keywords: Angioedema, C1 inhibitor, genital swelling, hereditary


How to cite this article:
García-Rodrigo CG, Maroñas-Jimenéz L, Menis D, Larráin H, Martínez LA. Acute and dramatic saxophone penis. Indian Dermatol Online J 2015;6:462-3

How to cite this URL:
García-Rodrigo CG, Maroñas-Jimenéz L, Menis D, Larráin H, Martínez LA. Acute and dramatic saxophone penis. Indian Dermatol Online J [serial online] 2015 [cited 2018 Dec 16];6:462-3. Available from: http://www.idoj.in/text.asp?2015/6/6/462/169726

We present the case of a 33-year-old white Spanish man with two days history of an asymptomatic fast-growing edema, first on both ankles and later on his penis. He denied any rash, urethral symptoms, or other systemic symptoms. The edema decreased on ankles after 24 h but not on the penis, where it began over the distal foreskin, progressing to the entire shaft and scrotum. was no inguinal lymphadenopathy. Due to extensive edema, the glans could not be visualised and the opening of the urethral meatus was blocked [Figure 1]. There was no history of unprotected sexual intercourse, trauma, insect bites, or previous similar episodes. However, he revealed that his maternal grandfather had recurrent limb edema, therefore, he and his mother were studied and diagnosed with a quantitative C1 inhibitor deficiency a year ago. We treated him with Berinert 500 U intravenous and after 5 h, the edema disappeared and no cutaneous pathology was observed on the glans.
Figure 1: Intense edema of the penis shaft and scrotum with no inguinal lymphadenopathy, erythema or skin lesions

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Saxophone penis refers to swelling and deformity of the penile shaft secondary to multiple causes. In case of acute male genital edema, it is essential to distinguish if the swelling is painful, which could be a potential emergency,[1] or if it is asymptomatic where hereditary angioedema (HAE) could be the rare cause. Angioedema may be hereditary, accounting for 5% of cases, or acquired by causes such as allergy, angiotensin-converting enzyme inhibitors, malignancies, autoimmunity, and connective tissue diseases. HAE is caused by a defect in C1 inhibitor, usually a quantitative deficiency, only 20% of it is because of a functionally impaired C1 inhibitor. It has an autosomal dominant inheritance. Although there is often a family history, a quarter of individuals may have a de novo gene mutation.[2] The first presentation of HAE most commonly affects the skin, usually edema of the extremities, face, or genitals. Patients can subsequently develop episodes of visceral edema -gastrointestinal and respiratory systems are the most frequently involved.

HAE is diagnosed by measuring the levels and functionality of serum C1 inhibitor; affected individuals also have low C4 levels. Acute episodes should be treated with intravenous purified C1 inhibitor concentrate or a bradykinin-2 receptor antagonist. For long-term prophylaxis, attenuated androgens or antifibrinolytic agents could be used.[3]

Including HAE in the differential diagnosis of genital swelling may prevent future potentially life-threatening episodes of visceral angioedema.

 
   References Top

1.
Weinberger LN, Zirwas MJ, English JC 3rd. A diagnostic algorithm for male genital oedema. J Eur Acad Dermatol Venereol 2007;21:156-62.  Back to cited text no. 1
    
2.
Pappalardo E, Cicardi M, Duponchel C, Carugati A, Choquet S, Agostoni A, et al. Frequent de novo mutations and exon deletions in the C1 inhibitor gene of patients with angioedema. J Allergy Clin Immunol 2000;106:1147-54.  Back to cited text no. 2
    
3.
Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med 2008;359:1027-36.  Back to cited text no. 3
    


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