By Gupta, Somesh; Ajith, C; Kanwar, Amrinder J; Sehgal, Virendra N; Et al
Summary: Genital elephantiasis is an important medical problem in the tropics. It usually affects young and productive age group, and is associated with physical disability and extreme mental anguish. The majority of cases are due to filariasis; however, a small but significant proportion of patients develop genital elephantiasis due to bacterial sexually transmitted infections (STIs), mainly lymphogranuloma venereum (LGV) and donovanosis. STI-related genital elephantiasis should be differentiated from elephantiasis due to other causes, including filariasis, tuberculosis, haematological malignancies, iatrogenic, or dermatological diseases. Laboratory investigations like microscopy of tissue smear and nucleic acid amplification test for donovanosis, and serology and polymerase chain reaction for LGV may help in the diagnosis, but in endemic areas, in the absence of laboratory facilities, diagnosis largely depends on clinical characteristics. The causative agent of LGV, Chlamydia trachomatis serovar L1-L3, is a lymphotropic organism which leads to the development of thrombolymphangitis and perilymphangitis, and lymphadenitis. Long-standing oedema, fibrosis and lymphogranulomatous infiltration result in the final picture of elephantiasis. Elephantiasis in donovanosis is mainly due to constriction of the lymphatics which are trapped in the chronic granulomatous inflammatory response generated by the causative agent, Calymmatobacterium (Klebsiella) granulomatis. The LGV- associated genital elephantiasis should be treated with a prolonged course of doxycycline given orally, while donovanosis should be treated with azithromycin or trimethoprimsulphamethoxazole combination given for a minimum of three weeks. Genital elephantiasis is not completely reversible with medical therapy alone and often needs to be reduced surgically.
Keywords: elephantiasis, lymphogranuloma venereum, donovanosis
Genital elephantiasis is one of the well-recognized clinical expressions of sexually transmitted infections (STIs), defined as massive enlargement of the genitals. The term ‘elephantiasis’ was originally used to describe the elephant-like appearance of the legs due to grotesque enlargement secondary to mechanical failure of the lymphatic system.1 Subsequently, the term was used to describe the similar enlargement of the arm, chest, breast, penis, scrotum, and vulva.2 Genital elephantiasis may occur due to a variety of infective and non-infective aetiologies causing lymphatic blockage in the genital region3-21 (Table 1).
Genital elephantiasis is unusual in areas where filariasis is not endemic. Bacterial STIs constitute one of the important causes of non-filarial genital elephantiasis in the tropics in particular. Penile venereal oedema, caused by gonococcal/herpes infection or scabies infestation,19 and penile friction oedema23,24 are transient, which resolve completely after treatment of the underlying cause, and may not lead to irreversible huge enlargement. However, the distinction between chronic, persistent, irreversible genital oedema and elephantiasis is arbitrary, and depends only on the subjective perception of size of the enlarged genitalia. Probably patients with long-standing irreversible genital oedema will end up with genital elephantiasis. The prime objective of the current dissertation is to bridge the gap in the contemporary literature pertaining to genital elephantiasis following STIs. Among the STIs, Chlamydia tmchomatis serovar L1-L3, responsible for lymphogranuloma venereum (LGV), and Calymmatobncterium (Klebsiella) granulomatis, a cause of donovanosis, are the most common causes of genital elephantiasis. In addition, genital elephantiasis has also been described as a complication of syphilis and infection with non- LGV strains of C. trachomntis.
Table 1 Genital elephantiasis: incriminating causes
Table 2 Genital elephantiasis: nomenclatures
Various nomenclatures are in use to describe genital elephantiasis based on the morphological appearance of the genitalia secondary to elephantiasis. The term ‘esthiomene’ is derived from a Greek verb which means to eat and carries forth the idea of something gnawed, eroded or ulcerated.25 In clinical terms, it refers to elephantiasic enlargement of the female genitalia along with ulcerations.3,26 Then do we have to look for another term to describe the similar condition in men? The definitions of the various terms used in relation to genital elephantiasis are given in Table 2.
History and epidemiology
Elephantiasis is one of the oldest, most bizarre and crippling diseases, and has a long history of worldwide distribution.28 Genital elephantiasis has been known since antiquity. Benjamin Neisius of the University of Strasburg, Germany in the year 1673, in his inaugural essay had described elephantiasis as an ‘Iliad of diseases’, occurring as complications of several diseases.28 The scientists and doctors, who had accompanied Napoleon to Egypt in the year 1798, described genital elephantiasis in a woman, and elephantiasis of the legs and genitals in a man (Figure 1). Since genital elephantiasis due to filariasis is quite uncommon in women, the case in question might have represented LGV-related elephantiasis.29 DominiqueJean Larrey, one of the French physicians accompanied the Napoleon’s army, had recognized the genital elephantiasis as a disease more of tropical climate than that of temperate climate. He wrote ‘they are endemic in hot climates; at least, they are seldom encountered in cold climates …’.30
Figure 1 A 30-year-old Egyptian woman (left) at the time of Napoleon Bonaparte, who had been afflicted with elephantiasis of external genitalia for several years. (Courtesy: Dr Terence M Russell and Dr Thomas G Russell, reproduced from reference )
Furthermore, he also had described the agony of patients due to enormousness of genital elephantiasis: ‘The malady inconveniences [the subject] only by the ponderosity [weight] and embarrassment that it manifests as it progresses – such that it obliges [the subject] to make use of some form of support.’ (From the text provided by Dr Terence M Russell and Dr Thomas G Russell, UK)
Genital elephantiasis due to STIs is an uncommon condition, and there are only sporadic reports that too from the tropical countries. The last large series of STI-related genital elephantiasis was published in early 1980s from India. In this study, 25 female patients with vulvar elephantiasis were described; 13 had LGV-associated elephantiasis and remaining 12 had donovanosis- associated pseudo-elephantiasis.31 LGV is endemic in several tropical and sub-tropical countries including West, Central and East Africa, India, Malaysia, Korea, Vietnam, South America, Papua New Guinea, and the Caribbean Islands32-35 across the globe. The proportion of genital ulcers that might be attributed to LGV varies from 1-10% in these areas. Perhaps, the lack of specific diagnostic criteria and the relatively poor degree of clinical suspicion of this condition might have prejudiced these estimates. HIV epidemic has led to outbreaks of LGV in Western Europe, mostly in men who have sex with men.36 Apparently, LGV occurs six times more frequently in men than in women. It probably remains under- or undiagnosed in women, because of the asymptomatic nature of the early lesions. Esthiomene, one of its late complications, has frequently been reported in women,31 whereas, penile and scrotal elephantiasis has been reported to occur in only 4% of LGV cases.37
While, pseudo-elephantiasis of the genitals, a complication of donovanosis, was first described by Nair and Pandalai38 in Indian patients. Donovanosis was prevalent in many parts of the world in the pre-antibiotic era. It has virtually disappeared from the developed world,39 though increased world travel and migration has resulted in limited outbreaks in industrialized nations.40 Current reports of the disease are only sporadic and largely confined to Papua New Guinea, India,41 South Africa (more common in Zulus), Caribbean islands, remote northern Australia (Aboriginal population), and Brazil. Genital elephantiasis due to donovanosis has largely been reported from India.38,41-43 However, occasional cases have also been reported from Australia44 and Papua New Guinea. 5 Ever since the advent of effective antibiotics, the incidence of elephantiasis due to donovanosis has recorded a perceptible decline from 24% in 1934,38 15-20% in 1954,4311% in 1966(46) and 5% in 1987,42 respectively in studies from India. The current literature seems to have depleted, re-affirming the impression that the disease hardly poses a challenge for the present. Reports of syphilis causing genital elephantiasis mostly appeared in the pre-penicillin era and no such cases have been reported in the indexed literature during the last quarter of the 20th century, though some cases of persistent chronic penile oedema due to syphilis leading to enlargement of the part have been reported recently.47
The lymphatic drainage from the anatomical site on the primary lesion probably determines the clinical picture of the disease. It is worthwhile to appreciate that the pathogenesis of elephantiasis caused by LGV and donovanosis are different, the synopsis of which is recounted below:
Lymphogmnuloma v\enereum (LGV)
The causative agent of LGV, C. trachomatis serovars L1-L3, is a lymphotropic organism which initiates the disease process primarily in the lymphatic channels, leading to thrombo-lymphangitis and peri- lymphangitis with an extension of the inflammatory process to the draining lymph nodes. The lymphangitis is marked by proliferation of endothelial cells lining the lymph vessels, and the lymph channels in the lymph nodes. The inflammation around the lymph nodes (peri- adenitis) might cause matting of adjacent lymph nodes. The latter is progressive, and in an untreated case abscesses may coalesce to form fistulae and tracts, confined to lymph nodes draining the site of primary infection. They might rapidly enlarge and undergo necrosis surrounded by densely packed endothelial cells.48 The inflammatory reaction might last for many months, and an eventual healing might take place by fibrosis. This may in turn destroy the normal structure of lymph nodes and obstruct the lymphatics. A combination of chronic oedema, sclerosing fibrosis, and active diffuse lympho- granulomatous infiltration in the subcutaneous tissue results in the final picture of massive enlargement of the genitalia.4 Fibrosis also compromises the blood supply which leads to the surface ulceration.48,51
The lymphoedema is mainly due to constriction of the lymphatics as they are trapped in the chronic granulomatous inflammatory response in the surrounding tissue.44 Later, healing with fibrosis further constricts the lymphatics and thus interferes with the lymphatic drainage of the area resulting in persistent lymphoedema and enlargement of the involved and dependent tissue. This is
sometimes referred to as ‘pseudo’ elephantiasis, since there is no direct involvement of the lymphatics and lymph nodes by the disease process.42
The lymphoedema associated with syphilis and chlamydial urethritis may be due to ‘scarring’ of the lymphatic channels as a result of persistent lymphatic insult due to a chronic inflammatory response.47
Genital elephantiasis, a physical disability, is a source of extreme mental anguish due to the ugly deformity. Sexual intercourse may not be possible and these morphological changes in the genitals may even interfere with walking.52 Clinical features of elephantiasis related to individual STIs are recounted below in the following text.
The clinical features of LGV might comprehensively be divided into three stages, namely the primary stage which identifies the site of inoculation, the secondary stage where there is an affliction of regional lymph nodes, and occasionally the anorectum, and the tertiary stage refers to the late complications affecting the rectum and genitalia, including elephantiasis. These late complications in the tertiary stage are frequent in women. Contrary to this, the late manifestations in men, apart from homosexuals, are fairly rare.49 In men, chronic inguinal lymphadenitis leads to penile and scrotal elephantiasis (Figure 2), approximately 1-20 years after infection. It may affect only the prepuce, shaft of the penis, and the scrotum alone or the entire male external genitalia. The genital tissues become woody indurated and often deformed, and the surface may become verrucous due to lymphangiectatic papules. The scrotum may reach a monstrous size.”3″4 The penis may become solidified and may assume the shape of a ‘ramrod’, ‘ram horn’ (Figure 3) or a saxophone.55 Erection is inhibited and sexual intercourse becomes impossible.
Figure 2 Early oedema of external genitalia associated with acute lymphadenitis in inguinal region in lymphogranuloma venereum. (Courtesy: Dr Kamal Aggarwal, Dr Sanjeev Cupta, and Dr Vijay K Jain. Reproduced from Sexually Transmitted Infections, Kumar B, Cupta S, eds. New Delhi: Elsevier, 2005)
In women, chronic progressive lymphangitis, and inguinal and pelvic adenitis may lead to chronic oedema, sclerosing fibrosis of subcutaneous tissue, elephantiasis and chronic genital ulceration, the ‘esthiomene’. It involves the labia, vulva and clitoris which gradually become enlarged. The size may vary from a mere tumefaction of the lips to large, pendulous, multilobed, unsightly masses of hypertrophied tissue hanging down and obstructing the vulval cleft56 (Figure 4). The oedema may extend from the clitoris to anus. The external surface of the labia majora, genitocrural folds, fourchette, urethral orifice, root of clitoris, and perineum may develop ulceration. These ulcers may be localized or they may be large and superficial or perforating. The vascular compromise is secondary to fibrosis, results in large, destructive ulcers or occasionally superficial ulcers with irregular, serrated edges and a shiny yellowish-white base. Genital elephantiasis is often associated with anorectal complications including fistulae and strictures both in men and women.
It is a chronic, progressively destructive, granulomatous infection of the superficial tissue of the genital region. The initial lesions of donovanosis are single or multiple subcutaneous nodules that erode through skin or mucosa to form an ulcer covered with “beefy’ red granulation tissue. Lymphoedema of the genital region is one of the long-term complications. However, in untreated cases, spontaneous healing may occur, but this is extremely slow with marked fibrosis, scar tissue contractures, and lymphatic obstruction that may produce the consequent elephantiasis like picture.57 As in LGV, elephantiasis in donovanosis is largely seen in women. Clinically, donovanosis-induced elephantiasis in women is characterized by firm, pedunculated, globular swellings with or without verrucous surface and de-pigmentation. It affects predominantly the labia majora (Figure 5) and the clitoris. More than one swelling may be present involving different areas of genitalia. The size of the elephantiasis is apparently not related to the duration of the disease process.41 There may be associated ulceration, especially in intertriginous areas.43 Pseudo- elephantiasis in men is extremely rare. Morphology of pseudo- elephantiasis involving the penis usually has gross, solid enlargement of distal part of the penis with the overlying skin being oedematous and thickened. Phimosis and overlying ulcerations may be present.43
Figure 3 Enlarged curved penis (Ram horn) with lymphagiectatic papules and inguinal scarring and sinuses in a patient with late stage of lymphogranuloma venereum
Figure 4 A huge swelling originating from clitoris in a woman with late stage of lymphogranuloma venereum (reproduced from reference )
Figure 5 Genital elephantiasis of labia majora due to donovanosis
Other sexually transmitted infections
A few reports describing syphilis as a cause of genital elephantiasis were the focus of attention in the pre-penicillin era. It is difficult to confirm whether the elephantiasis was due to syphilis itself or due to other co-existing diseases like tuberculosis and LGV. Elsahy5 emphasized that syphilis plays a dominant role in these cases. Recently a case of genital elephantiasis occurring several years after untreated chlamydial urethritis has been reported, which responded to long-term doxycycline therapy.6
Diagnosis and differential diagnoses
Genital elephantiasis is a significant medical problem and persons affected may become a major burden to their family and community, especially when the disease interferes with their economic livelihood. Early diagnosis of the underlying conditions and appropriate management may prevent the development of these deformities. In a patient with genital elephantiasis, importance of a proper history and thorough clinical examination cannot be overemphasized, as it may help to find the cause for the elephantiasis. Computerized tomography may detect pathologies in the anorectum and provide supportive evidence for LGV (Figure 6). When the cause of lymphoedema is suspected to be due to a sexually transmitted pathogen, then patients should be investigated for LGV and donovanosis. Nucleic acid amplification test for donovanosis offers highest sensitivity, but is not commercially available.58 Moreover, its value in late cases of genital elephantiasis is not known. If active granulomatous ulcers are present, demonstration of the donovan bodies with bipolar staining in large foamy mononuclear cells by microscopy from lesion scrapping is the mainstay of diagnosis. The diagnosis of LGV-associated elephantiasis is supported by high titre serology and identification of the organism in pus or bubo fluid by cytology or culture. The pathogen is likely to be isolated from the purulent discharge even in the late cases of LGV-associated elephantiasis.50 Micro-immunofluorescence test is the only serological means of distinguishing LGV strains of C. trachomatis from other serovars,59 though it is still not in routine use as it requires a fluorescent microscope and a trained technician. Only high titres (>1:128) are specific for LGV. Polymerase chain reaction (PCR) amplification and sequence analysis of the omp 1 gene, which encodes the major outer membrane protein (MOMP), has also been found to be useful in identification of LI-L3 serovars.60 Nevertheless, diagnosis of LGV and donovanosis is primarily clinical, more so in endemic areas where diagnostic laboratory facilities are not easily available. Some clinical features may help in differentiating elephantiasis caused by LGV and donovanosis (Table 3).61
Figure 6 Contrast enhanced computerizd tomography scan of the pelvis of a patient with anorectal-genital LCV showing mural thickening of the rectum with presence of perirectal staunching. There is loss of the fat plane between the rectum and the left seminal vesicle. The patient is same as shown in Figure 7. (Reproduced from Sexually Transmitted Infections, Kumar B, Cupta S, eds. New Delhi: Elsevier, 2005).
Table 3 Genital elep\hantiasis: differentiating features between caused by LGV and donovanosis61
Elephantiasis associated with tuberculosis is not uncommon in tropics and may closely resemble elephantiasis due to LGV. Both are associated with inguinal lymphadenitis, though reactive Mantoux test, suggestive histopathology, isolation of Mycobacterium tuberculosis by PCR or culture, and response to anti-tubercular therapy are confirmatory for tuberculosis.7,8 Elephantiasis due to filariasis is seen primarily in men living in endemic areas, but occasional cases in women have been reported.62 Demonstration of peripheral blood eosinophilia, and microfilariae in nocturnal blood samples or lymph node aspirates is diagnostic.62 In the western world, the majority of genital elephantiasis are from trauma or surgery to remove gynaecological, urological, abdominal or prostatic cancers or radiotherapy to the lymph nodes in the groin, and a careful history will help in the diagnosis. Primary lymphoedema affecting only the genitals may be noticed from birth or during the teens. Lymphanogiography is useful to distinguish between primary or secondary lymphoedema. In a minority of patients, the diagnosis of the underlying condition will remain uncertain.63
Treatment modalities of genital elephantiasis due to STIs require an interdisciplinary approach involving genitourinary medicine physicians, urologists, and dermatologists. The objectives of treatment are to reduce swelling, restore shape and normal sexual function, and prevent inflammatory episodes, e.g., recurrent cellulitis.64
Genital elephantiasis is usually irreversible. Physical methods (i.e. compression therapy), drugs, and surgery are three mainstays of therapy available for lymphoedema. Compression therapy is not practical for lymphoedema of the genitalia for obvious reasons; however, minor swelling can be treated with support hosiery. Medical therapy should be the first line of treatment and any surgical intervention should be undertaken only under the cover of appropriate antibiotics. In case of genital elephantiasis caused by LGV, doxycycline lOOmg twice daily should be given for prolonged periods (improvement reported with up to 13-month therapy6) (Figures 7a and b). In cases of donovanosis, azithromycin 1 g a day, followed by 500 mg daily or trimethoprim 80 mg and sulphamethoxazole 400 mg two tabs twice a day should be given till ulcerations (if present) heal. If there is no ulceration, a two-week therapy covering surgical reconstruction is recommended.44 Doxycyline 100 mg twice daily, ciprofloxacin 500 mg twice daily, or erythromycin base 500 mg four times daily are other alternatives.
Surgery is the only effective option for a select group of patients in whom the disorder is disabling and persistent. Operations fall into two categories: bypass procedures and reduction procedures.65
A number of procedures like omental pedicle, the skin bridge, anastomosing lymph nodes to vein, and more recently, lymphovenous anastomosis with the aid of an operating microscope are described. However, poor long-term results of these technically challenging procedures have made them unpopular worldwide.
Figure 7 (a) Saxophone penis in a homosexual man with tertiary stage of LCV. Prominent lymphangiectatic papules leading to pebbly surface are evident (reproduced from reference ). (b) After one- year therapy with doxycycline, there is complete subsidence of scrotal swelling and significant reduction in the swelling of penile shaft, though swelling of prepuce is still persisting due to its dependent position
Two types of reduction procedures are commonly performed: (1) removal of the subcutaneous elephantoid tissue with preservation of the overlying skin for covering the penis and denuded testes, and (2) radical excision of the skin and subcutaneous tissue. Following this, scrotal reconstruction can be achieved by free skin graft, positioning a skin flap from between the scrotum and inguinal area using skin of the scrotal neck alone or supplemented with a rotation flap from the skin of the thigh.
Denuded penis is best covered by split thickness skin graft which is sutured to the dorsal, ventral and coronal side of the penis along with fixation to the root of it in the form of a Z to avoid circular contracture and a longitudinal scar on the shaft of the penis.
Labial reduction is easily achieved by wide elliptical excision with a single suture line.31 Recurring swelling in a minority group will be benefited by another similar procedure. Larger labial defects may be covered with myocutaneous, floating island or fasciocutaneous flaps.66
There are no studies available demonstrating the long-term efficacy of reduction procedures in STI-related genital elephantiasis. Chakravarty and Rajagopalan : found no recurrence at two years in 16 women with sexually transmitted disease (STD)- related esthiomene treated with reduction procedures. Burnard et al.65 reported long-term followup of 41 patients (31 male and 10 female) with genital lymphoedema, mostly of filarial origin. They showed complete ‘cure’ lasting at least 10 years in 57% of men and two-thirds of women.65 Dandapat et al.52 reported a recurrence rate of 7.13% in a large series of 350 male patients with genital elephantiasis due to filaria. McDougal20 reported no recurrence after a follow-up of six months to 10 years in male patients with non-STI-related genital lymphoedema treated with reduction procedures.
Erysipelas is a frequent complication in patients with genital elephantiasis, as the defence mechanism of the skin is impaired due to chronic lymphatic obstruction and compromised blood supply. Repeated episodes of erysipelas may further increase the lymphatic obstruction and elephantiasis.10 Such patients often benefit from long-term benzathine penicillin therapy and vaccination against streptococci.10 Meticulous skin care is important to prevent recurrent erysipelas.
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(Accepted 21 April 2005)
Somesh Cupta MD DNB1 C Ajith MD1, Amrinder J Kanwar MD MNAMS1 Virendra N Sehgal MD FRAS2, Bhushan Kumar MD MNAMS1 and Uttam Mete MS MCh3
1 Department of Dermatology and Venereology, Chandigarh; 2Department of DermatoVenereology (Skin/VD) Centre, Sehgal Nursing Home, Panchwati, Azadpur, Delhi; 3Department of Urology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Correspondence to: Dr Somesh Cupta, Department of Dermatology and Venereology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India
Email: [email protected]
Copyright Royal Society of Medicine Press Ltd. Mar 2006