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lymphogranuloma venereum (inguinale); Favre-Durand-Nicholas disease; tropical bubo

Etiology: -> Chlamydia trachomatis (serovars L1, L2, & L3) Epidemiology: 1) 3rd decade, while most sexually active 2) most cases in non whites, but probably no racial difference 3) acute infection is more common in males 4) anogenitorectal syndrome more common in females & homosexual men 5) sporadic in North America, Europe, Australia & most of Asia & South America 6) endemic in east & west Africa, parts of southeast Asia, parts of South America & the Caribbean Pathology: 1) chlamydia in purulent exudate is inoculated onto skin or mucosa of sexual partner & gains entry through minute lacerations & abrasions 2) incubation period 3-12 days for primary stage; 10-30 days for secondary stage 3) lymphatic obstruction of genitalia may occur 4) perirectal abscesses 4) fistulas Clinical manifestations: 1) primary stage: a) painless papule, abrasion or ulceration at the site of inoculation b) most patients do not present with this finding c) lesions resolve without treatment 2) secondary stage a) systemic manifestations 1] headache 2] malaise 3] fever 4] arthralgias b) local manifestations 1] inguinal syndrome a] inguinal lymphadenopathyabove & below the inguinal ligament [4] b] bubo near the inguinal ligament c] lower abdominal & back pain 2] anogenitorectal syndrome a] draining fistulas b] proctitis c] tenesmus & rectal pain d] perianal condylomata c) systemic skin manifestations 1] erythema nodosum (10%) 2] scarlatiniform eruptions 3] urticaria Laboratory: 1) positive skin test with Frei antigen 2) serology: a) positive complement fixation test with Frei antigen b) serovars L1, L2, & L3 specific testing 3) culture bubo aspirate Differential diagnosis: 1) primary stage a) genital herpes b) primary syphilis c) chancroid 2) inguinale syndrome a) incarcerated inguinal hernia b) plague c) tularemia d) tuberculosis e) genital herpes f) syphilis g) chancroid h) Hodgkin's disease 3) anogenitorectal syndrome a) rectal stricture due to rectal cancer b) trauma c) actinomycosis d) tuberculosis e) schistosomiasis f) inflammatory bowel syndrome 4) esthiomene a) filariasis b) mycosis 5) hidradenitis suppurativa if all lesions could be associated with hair follicles [6] Complications: 1) rectal strictures & fissures [4] 2) elephantiasis of the genitals (esthiomene) a) generally in females b) may ulcerate c) may occur 20 years after primary infection Management: 1) prevention: condom use 2) antimicrobial therapy a) cures infection, prevents ongoing tissue damage b) tissue reaction & residual scarring may occur c) doxycycline* 100 mg PO BID for 21 days d) erythromycin 500 mg PO QID for 21 days e) sulfisoxazole 500 mg PO QID for 21 days or Bactrim DS 1 PO BID for 21 days 3) sexual partners should be referred for evaluation even if the partners have no symptoms - 100 mg of doxycycline BID x 7 days, - 1000 mg azithromycin (single dose) 4) buboes may require aspiration or incision & drainage * treatment of choice

Related

bubo Chlamydia trachomatis Frei test

General

granulomatous disease sexually-transmitted disease; sexually-transmitted infection; venereal disease (STD, STI)

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 908
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
  3. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 896-898
  4. Prescriber's Letter 12(8): 2005 Lymphogranuloma Venereum Detail-Document#: 210820 (subscription needed) http://www.prescribersletter.com
  5. Martin-Iguacel R, Llibre JM, Nielsen H et al Lymphogranuloma venereum proctocolitis: a silent endemic disease in men who have sex with men in industrialised countries. Eur J Clin Microbiol Infect Dis. 2010 Aug;29(8):917-25 PMID: 20509036
  6. NEJM Image Challenge: http://www.nejm.org/image-challenge