Saxophone penis associated with penoscrotal lymphangiomatosis
Ahmad Al Aboud1, Mohammad Hakim2, Ibrahim Alehibi2, Homaid Alotaibi2, Farees Al Saadi2, Yusra Bindogji2, Latifah Algithami4, Saafa Alabsi2, Abdulmajid Algithami3, Fahad Algithami3, Foud Algithami4, Hussain Khobrani4, Ayman Alkhatabi4, Aydhah Alhesaini4, Sami Al Mehmadi5, Waleed Alqurashi5, Adnan Khairallah5, Khalid Al Aboud2
1Department of Dermatology, King Abdullah Medical City, Makkah, Saudi Arabia, 2Department of Dermatology, King Faisal Hospital, Makkah, Saudi Arabia, 3Department of Infection Control, King Faisal Hospital, Makkah, Saudi Arabia, 4Department of Physiotherapy, King Faisal Hospital, Makkah, Saudi Arabia,5 Department of Radiology, King Faisal Hospital, Makkah, Saudi Arabia
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Lymphangiomatosis is a condition marked by the presence of cysts that result from an increase both in the size and number of thin-walled lymphatic channels that are abnormally interconnected and dilated. It can affect skin and other organs in the body. Genital lymphangiomatosis may arise from primary or secondary lymphatic abnormalities.It affects both sexes, and frequently misdiagnosed as genital warts. In this concise report, we present an adult male with diffuse penoscrotal lymphangiomatosis associated with a peculiar penile deformity, known as “Saxophone penis”.
Key words: Genital swelling; Lymphangiomatosis; Sexually Transmitted Diseases (STDs); Warts
Prescence of multiple lymphangiomas in the genital area, otherwise known as genital lymphangiomatosis, can primary (due to lymphatic malformation) or secondary to disorders that block or interfer with the drainage of lymph in this area [1–13].
The primary genital lymphngiomatosis does not usually present at birth (congenital), and often, present later in life.
Here, we present an adult male with diffuse penoscrotal lymphangiomatosis associated with a peculiar penile deformity, known as “Saxophone penis”.
A-60 years -old Afghani male patient, not known to have a chronic medical problems before, who presented with persistent and progressive, multiple papules on penis and scrotum for more than 15 yearys duration.There is mild itching but no pain.
The penis is swollen and deformed (Fig. 1a), giving rise to the so called ‘ramrod’, “ram horn penis,” or ‘saxophone penis’.Simulated to the saxophone, which is a widely-known musical instrument (Fig. 1b).
He denied any urethral symptoms, genital ulcers, or other systemic symptoms.There was no history of unprotected sexual intercourse, trauma, operations or any things to suggest a disorders of secondary genital lymphedema.
He is diagnosed several times before as genital warts.
All routine tests including Mantoux test, chest X-ray, ultrasound of abdomen, serum Venereal Disease Research Laboratory (VDRL) test were within normal limits. The patient was seronegative for HIV-1 and 2.
Punch skin biopsy confirmd the diagnosis of lymphangiomatosis and the patient is refered for urologist for further evaluation.
Cutaneous genital lymphangiomatosis is not a Common condition [1–13].
Lymphangiomatosis of primary cause, like other lymphatic malformations, is thought to be the result of congenital errors of lymphatic development occurring prior to the 20th week of gestation.
The lymphangiomatosis and penile deformity in our case is most likely due to primary isolated lymphadema, which is consequnce of genital lymphatic malformation.
Disorders which may precipitate secondary genital lymphedema include filariasis and sexually transmitted diseases (STDs) like lymphogranuloma venereum (LGV), surgical removal of inguinal lymph nodes .
Although lympahtic malformations and lymphangiomatosis are technically benign, these deranged lymphatics tend to invade surrounding tissues and cause problems due to invasion and/or compression of adjacent structures. As seen in our case, it precipitate anatomical deformity.
Scrotal lymphatic malformation with absent corpora cavernosa has been also reported .
“Saxophone penis” refers to swelling and deformity of the penile shaft secondary to multiple causes. It is a term used when the penis gets twisted along its long axis, giving it the appearance resembling a saxophone.
The latter, referred to colloquially as the sax, is a widely-known musical intrument.It is type of single-reed woodwind instrument with a conical body, usually made of brass.
The cause for this shape is not well understood; however, it is likely that the peculiar penile anatomay makes any fluid or vascular overload in the soft tissue of the penis to render the penis to this shape .
The different conditions causing a saxophone penis, reported in the literature, are primary lymphedema , penile tuberculosis , lymphogranuloma venereum in its tertiary stage , topical imiquimod use , entomophthoromycosis (basidiobolomycosis) , paraffin injections , and hereditary angioedema (HAE) .
In case of acute male genital edema, it is essential to distinguish if the swelling is painful, which could be a potential emergency, or if it is asymptomatic where HAE could be the rare cause .
It is difficult to acheive a complete cure for lymphatic malformations with different therapeutic modalities, including surgery, and its managment remain to be a challenge.
Physicians in genreral and dermatologists, venereologists and STDs specialists in particular, need to keep lymphangiomatosis in their differentials for genital lesions in order not to misdagnose it as warts .
The examination of the patient was conducted according to the principles of the Declaration of Helsinki.
The authors certify that they have obtained all appropriate patient consent forms, in which the patients gave their consent for images and other clinical information to be included in the journal. The patients understand that their names and initials will not be published and due effort will be made to conceal their identity, but that anonymity cannot be guaranteed.
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