The genital elephantiasis is more commonly encountered in tropical and subtropical countries like India [5]. These regions are endemic in filariasis, and lymphatic alterations are mainly due to the infestation by Wuchereria bancrofti. Almost 20% of male population can be affected [5].
Outside of these regions, genital elephantiasis represents a diagnostic challenge. McDougal made a classification of lymphedema of external genitalia which is based on factors such as congenital versus acquired, sporadic versus inherited and age of onset [4]. Identifying the underlying etiology is mandatory because, depending on the cause of the penoscrotal edema, conservative measures such as medical treatment against lymphogranuloma venereum and bancroftian filariasis are helpful and can cure early stages of the disease [6].
In non-endemic countries with filariasis, the neoplastic and congenital origins must be suspected first. Otherwise, the genital elephantiasis is idiopathic or due to infectious diseases. Indeed, the causative agent of lymphogranuloma venereum (LGV), Chlamydia trachomatis serovar L1-L3, is a lymphotrophic organism which leads to the development of severe alterations in lymphatic channels. Therefore, LGV can be complicated by penoscrotal elephantiasis [2, 7], and 4% of patients with LGV have been reported to develop penile and scrotal elephantiasis as a late complication [8]. Early anti-infectious therapy with doxycycline given orally is important to prevent genital elephantiasis in these cases [1].
Donovanosis, another sexually transmitted infection due to Klebsiella granulomatis, can generate in 5% of patients elephantiasis [9, 10]. Donovanosis should be treated with azithromycin or trimethoprim–sulfamethoxazole combination for a minimum of three weeks
Syphilis should also be suspected as a differential diagnosis especially in patients with systemic symptoms accompanied by penoscrotal elephantiasis with a history of genital ulcer [11].
Other inflammatory or infectious diseases are described to cause genital lymphedema such as: recurrent streptococcus infections with resultant post inflammatory changes [2, 12] and hidradenitis suppurative which is a chronic recurrent inflammation of apocrine sweat glands [3]. However, although performing thorough serologic and radiographic evaluation, in many cases no etiology is identified and the elephantiasis is classified as idiopathic.
For advanced stages surgery remains the only solution. Complete excision of all lymphedematous tissue, with preservation of the testes, spermatic cord and the penis, followed by plastic penoscrotal reconstruction is appropriate to avoid recurrence.