Lymphogranuloma Venereum

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(Climatic bubo; lymphogranuloma inguinale; lymphopathia venereal; Durand-Nicolas-Favrq Disease)

Introduction

  • A chronic disease caused by Chlamydia trachomatis (serotypes L1,  L2, L3), an obligate intracellular microorganism
  • Most common in Asia, Africa, and South America
  • In Europe and North America, it is most prevalent among homosexuals, immigrants from endemic areas and people returning from endemic areas, such as soldiers, seamen, and vacationers

Clinical features

  • A chronic granulomatous, locally destructive disease that is characterized by progressive, indolent, serpiginous ulceration of the groins, pubes, genitals and anus
  • May be classified into primary, secondary, and late stages

Primary stage

  • After an incubation period of 7 - 15 days, a papule or small non-indurated painless ulcer appears - Usually goes unnoticed
  • Extra-genital lesions (rectal, oral) have also been described
  • Women probably act as asymptomatic carriers
  • Patients are very rarely seen at the primary stage

Secondary stage

  • About 3 - 6 weeks post-contact a uni-or bilateral massive inguinal lymphadenopathy (bubo) appears
  • The glands elongate along the Poupart's ligament to become sausage shaped
  • Buboes progress to involve the glands above and below the ligament, so that the depression formed by the ligament which separates these two groups of glands gives the "sign of the groove"
  • Pain in the gland is usual, and as the glands are matted together, the overlying skin develops an erythematous or violaceous hue
  • The glands eventually become fluctuant, break down and discharge
  • Inguinal lymphadenopathy occurs in only 20 - 30% of women with LGV
  • There is primary involvement of the rectum, vagina, cervix, or posterior urethra, which drain to the deep iliac or perirectal nodes
    • This may produce symptoms of lower abdominal or back pain
  • Systemic symptoms usually present with:
    • Fever
    • Malaise
    • Arthritis
    • Loss of weight
  • Skin manifestations (erythema nodosum, papulo-pustular lesions and photodermatosis)
    • Raised ESR

Late stage

  • Spontaneous remission is common, though some patients enter the late stage
  • Characterized by disfiguring and destructive sequelae
  • Impairment of the lymphatic drainage from fibrotic scarring leads to distant oedema and gross elephantiasis of the genitalia
    • There could be associated anorectal and vaginal strictures

Complications

  • Systemic spread of C. trachomatis in the secondary stage resulting in arthritis, pneumonia, hepatitis or rarely perihepatitis
  • Other rare systemic complications include pulmonary infection, cardiac involvement, aseptic meningitis, and ocular inflammatory disease
  • The late stage may be complicated by the genito-anorectal syndrome
    • Reported more in homosexual men, and women who engage in receptive anal intercourse
  • Patients may also complain of fever, pain, and tenesmus. Obstructed labour from elephantiasis of the vulva

Differential diagnoses

Buboes:

  • Chancroid; Infections of the lower limbs; Hodgkins disease and other lymphomas; Plague

Tularemia Late stage:

  • Tuberculosis; Deep mycosis of the genitalia; Squamous cell or basal cell carcinoma

Investigations

  • Culture and cell typing of the isolate from an aspirate of involved lymph node
  • Serological tests e.g. CFT and MIF; PCR

Treatment goals

  • Same as for gonorrhoea

Drug treatment

Recommended  regimen:

Doxycycline - 100 mg orally every 12 hours for 14 days

Or:

Erythromycin - 500 mg orally every 6 hours for 14 days

Alternative regimen:

Tetracycline - 500 mg orally every 6 hours for 14 days

Adjuvant measures

  • Aspirate fluctuant lymph nodes through healthy skin
  • Incision and drainage or excision of nodes may delay healing and is not recommended
  • Some patients with advanced disease may require treatment for longer than 14 days, and sequelae such as strictures and/or fistulae may require surgery

Notable adverse drug reactions,  contraindications and caution

Prevention

  • Counselling, Compliance, Condom use and Contact treatment