INTRODUCTION

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”.

CASE REPORT

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).

Figure 1: (a) Saxophone penis associated with penoscrotal lymphangiomatosis. (b) The saxophone.

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.

DISCUSSION

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 20^th 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 [7].

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 [13].

“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 [6].

The different conditions causing a saxophone penis, reported in the literature, are primary lymphedema [4], penile tuberculosis [5], lymphogranuloma venereum in its tertiary stage [7], topical imiquimod use [8], entomophthoromycosis (basidiobolomycosis) [9], paraffin injections [12], and hereditary angioedema (HAE) [10].

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 [10].

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 [1].

Notes