Letter to Editors
Esthiomene: An unusual presentation of elephantiasis
Anand Pai1, Umadevi V2, Narayanasamy S3
1MBBS, Resident Doctor, Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Pondicherry , INDIA.
2MD, Professor, Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Pondicherry, India.
3MD, Assistant professor, Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Pondicherry, India.


Address correspondence to:
Anand Pai
Department Of General Medicine
Aarupadai Veedu Medical College and Hospital
Kirumampakkam, Pondicherry - 607402
Phone: 919841320712, Ph: 044-28342171
Email: cdrocks_87@yahoo.co.in

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Pai A, Umadevi V, Narayanasamy S. Esthiomene: An unusual presentation of elephantiasis. International Journal of Case Reports and Images 2012;3(9):57–59.

Letter to Editors

To the Editors,

Genital elephantiasis is an important medical problem in the tropics as it is associated with physical disability and extreme mental anguish especially in females. Genital elephantiasis is a very rare condition and due to prevalence of sexually transmitted infections (STIs) it is even rarer. Elephantiasis and chronic genital ulceration in women is called Esthiomene'. [1]

A 40-year-old married female, mason by occupation, presented with swelling of the vulva and genital ulcer for the past five years. She complained of difficulty in micturition and difficulty in walking. She also had history of painful swellings in the inguinal region, on both sides, for the past seven years. There was no history of fever, cough with expectoration or weight loss. Her menstrual cycles were regular.

She had two healthy children. She gave a history of her husband's extramarital contacts. On genital examination two large nodular swellings of size 15×7×5 cm were present involving both the labia majora (Figure 1). Multiple nodules were present on the mons pubis. Multiple superficial and deep ulcers of varying size were present on the inner aspect of both labia majora normal. Hemogram, LFT and renal parameters were normal. Blood VDRL was non reactive. Serology for HIV antibodies and filariasis was found to be negative. Smear for malarial parasite and microfilaria was negative. Tissue smear for Donovan bodies was negative. Albumin/Globulin ratio was reversed (0.6). Tissue biopsy of the swelling was taken and histopathological study revealed lymphangiectasia (Figure 2). Culture of discharge in McCoy's medium showed growth of lymphogranuloma venereum strains. Polymerase chain reaction study showed features specific for Chlamydia trachomatis L1-L3 serovars which confirmed the diagnosis. The patient was started on doxycycline 100 mg twice daily for 15 days and was referred to plastic surgery. As no major structures were involved excision of the swelling was done. Postoperatively patient was followed up and had no further complaints.

Among sexually transmitted infections, lymphogranuloma venereum and donovanosis are the most common, others being syphilis and infection with non-LGV strains of Chlamydia trachomatis. Chlamydia trachomatis serovars L1-L3 are the causative agent of LGV and initiates the disease process primarily in the lymph channels leading to thrombolymphangitis and perilymphangitis. [2] Extension of inflammatory process to draining lymph nodes causes periadenitis, matting of lymph nodes, formation of abscesses, fistulae and sinuses. Healing takes place by fibrosis. A combination of chronic oedema, sclerosing fibrosis and active lymphogranulomatous infiltration in the subcutaneous tissue results in the massive enlargement of genitalia. These late complications occur in the tertiary stage of LGV and are frequent in women. The diagnosis of LGV associated elephantiasis is supported by high titre serology and identification of the organism in the pus or bubo fluid by cytology or culture. [3] Chlamydia trachomatis is a obligatory intracellular organism and diagnosis is usually made by tissue culture on McCoy's medium, direct immunofluoresence and polymerase chain reaction. Microimmunofluoroscence test is the only serological means of distinguishing LGV strains of Chlamydia trachomatis from other serovars. Polymerase chain reaction amplification and sequence analysis of the omp 1 gene is also useful in identification of L1- L3 serovars. [4] History of exposure to infection, a small transitory primary lesion, followed by chronic inguinal adenitis resulting in typical suppuration and fistulation is characteristic of LGV. With the exclusion of tuberculosis, filariasis, Hodgkin's disease, gonorrhoea, syphilis, malignant disease and chancroid, the diagnosis of the condition becomes at once easy.

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. 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 100 mg twice daily should be given for prolonged periods (improvement reported with up to 13 month therapy). Surgery is the only effective option for a select group of patients in whom the disorder is disabling and persistent. Reduction procedures include labial reduction which is easily achieved by wide elliptical excision with a single suture line. [5] [6] Recurring swelling in a minority of patients will be benefited by another similar procedure. Larger labial defects may be covered with myocutaneous, floating island or fasciocutaneous flaps. [7] However, there are no studies available demonstrating the long-term efficacy of reduction procedures in STI-related genital elephantiasis. Due to the manifold aspects of the disease and of the possibility of the lesions remaining insignificant for a long time, every case of genital elephantiasis or rectal stricture needs to be subjected to a critical investigation in regard to the possibility of this specific infection. Hence we found it worthwhile to report this case.

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Figure 1: Vulval elephantiasis with chronic genital ulcerations

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Figure 2: Histopathology of the vulval swelling showing lymphangiectatic changes (H&E, x400).

  1. Gupta S, Gupta U, Gupta DK. A gigantic esthiomene. Indian J Sex Transm Infect 1997;18:75–6.    Back to citation no. 1
  2. Smith EB, Custer RP. The histopathology of lymphogranulomavenereum. J Urol 1950;63:546.    Back to citation no. 2
  3. Sevinsky L, Lambierto A, Casco R, Woscoff A. Lymphogranuloma venereum:tertiary stage. Int J Dermatol 1997;36:47–9.   [Pubmed]    Back to citation no. 3
  4. Jurstrand M, Falk L, Fredlund H, et al. Characterization of Chlamydia trachomatis omp 1 genotypes among sexually transmitted disease patients in Sweden. J Clin Microbiol 2001;39:3915–9.   [CrossRef]   [Pubmed]    Back to citation no. 4
  5. Burnard K, Mortimer P, Browse N. Management of genital lymphoedema In: Browse N, Burnard KG, Mortimer PS, eds. Diseases of the lymphatics. London: Arnold 2003;217–30.    Back to citation no. 5
  6. Chakravarty A, Rajagopalan G. Surgical treatment of vulvar esthiomene. Indian J Sex Transm Dis 1981;2:49–51.    Back to citation no. 6
  7. Prakash S, Radhakrishna K. Problematic ulcerative lesions in sexually transmitted diseases: surgical management. Sex Transm Dis 1986;13:127–33.   [Pubmed]    Back to citation no. 7
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Author Contributions:
Anand Pai – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Umadevi V – Conception and design, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Narayanasamy S – Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Guarantor of submission:
The corresponding author is the guarantor of submission.
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Conflict of interest:
Authors declare no conflict of interest.
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