Fournier gangrene N49.8

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Lydia König

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Acute ulcerative genital gangrene; Erysipelas gangraenosum genitalium; Fournier gangrene; Fournier Gangrene; Fournier's gangrene; Gangraena acuta genitalium; gangraenosum ulcer; genital gangrene acute ulcer

History
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Baurienne, 1763; Fournier, 1883

Definition
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Life-threatening, fulminant, necrotizing inflammation of the male/female genitals, perineum or perianal region The clinical picture corresponds to a foudroyant necrotizing fasciitis and requires immediate urological or proctosurgical (possibly intensive care) intervention.

Etiopathogenesis
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Unclear, possible infection with streptococci ( Erysipelas gangraenosum genitalium, Erysipelas genitalium), fusiform bacteria, spirochetes etc. Possibly Sanarelli-Shwartzman phenomenon. Predisposing factors discussed are previous surgery or trauma (e.g. also after dental piercing: Ekelius L et al. 2004) and systemic diseases (e.g. diabetes mellitus).

In summer 2018 the FDA has included Fournier gangrene as a rare side effect in the technical information of SGLT2 inhibitors. A connection with therapeutically intended glucosuria is suspected.

Manifestation
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  • m:w=2:1
  • Age: between the 30th and 80th LJ.
  • Average age is given in larger studies as 59 years.
  • Less frequent in adolescents, children and infants.

Localization
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Perianal 40%; genital region: 40%;

Clinical features
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Sudden febrile onset, acute, very painful redness and swelling. Blistering with serous and haemorrhagic content, formation of a rapidly expanding necrosis, destruction of the skin of the penis, including the corpora cavernosa and the scrotal skin. Possibly exposed testicles. Mostly severe general complications up to septic shock.

Histology
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Mostly a compact tumour penetrating the entire dermis with nests and strands of oval to polygonal, distended cells with eosinophil granulated cytoplasm. The cell nuclei are often pynotic. A characteristic feature of granular cell tumors is a distinctly acanthotic epidermis.

Immunohistochemistry: Tumor cells are positive for S100, neuron-specific enolase and myelin basic protein.

Differential diagnosis
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General therapy
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Immediate transfer to surgical intensive care unit! If necessary, surgical therapy with generous necrotic excision and drainage, temporary transfer of the testicles into a subcutaneous thigh pocket. After healing plastic reconstruction.

Internal therapy
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  • High-dose parenteral therapy with broad-spectrum antibiotics such as ampicillin (e.g. Binotal). Adults: 150-200 mg/kg bw/day, children > 1st year of life 100-400 mg/kg bw/day distributed on 4 ED, infants < 1st week of life 50-150 mg/kg bw/day distributed on 2-3 ED, infants > 1st week of life 100-300 mg/kg bw/day distributed on 3-4 ED.
  • Alternative: benzylpenicillin (e.g. penicillin Grünenthal), adults: 6 times 4 million IU/day, children > 1 year of age: 50,000-250,000 IU/kg bw/day divided into 4-6 days, infants < 1 week of life: 50,000-250,000 IU/kg bw/day divided into 2 days, infants > 1 week of life 75,000-350,000 IU/kg bw/day divided into 4 days.
  • Alternative: gentamicin (e.g. refobacin) Adults: 3-5 mg/kg bw/day divided into 1-3 doses, children > 1st year of life: 5-7 mg/kg bw/day divided into 3-4 doses, infants < 1st week of life: 5 mg/kg bw/day divided into 2 doses, infants > 1st week of life: 7.5 mg/kg bw/day divided into 3 doses combined.
  • For penicillin resistance: 3rd generation cephalosporins, e.g. cefotaxime (claforan) 3 times/day 2 g i.v.
  • In case of resistance to therapy: combination of a cephalosporin with an aminoglycoside, e.g. rocephin 2 g/day i.v. with refobacin 1 time/day 240 mg i.v. or combination of vancomycin (e.g. Vanco-Cell) 2 time/day 1 g i.v. with gentamicin (see above).

Progression/forecast
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The lethality rate is 20-30%.

Literature
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  1. Bonner C et al (2001) Fournier gangrene as a rare complication after stapler hemorrhoidectomy. Case report and review of the literature. Surgeon 72: 1464-1466
  2. Eke N et al (2000) Fournier's gangrene: a review of 1726 cases. Br J Surg 87: 718-728
  3. Ekelius L et al (2004) Fournier's gangrene after genital piercing. Scand J Infect Dis 36:610-612.
  4. Fournier JA (1883) Gangrène foudroyante de la verge. La semaine médicale (Paris) 3: 345
  5. Pediment GD (2002) Comment on Ch. Bonner et al.: Fournier gangrene as a rare complication after stapler hemorrhoidectomy. Surgeon 73: 288
  6. https://www.aerzteblatt.de/nachrichten/97537/Diabetes-FDA-warnt-vor-Fournier-Gangraen-durch-SGLT2-Inhibitoren
  7. https://www.fda.gov/Drugs/DrugSafety/ucm617360.htm
  8. Kilic A (2001) Fournier's gangrene: etiology, treatment, and complications. Ann Plast surgery 47: 523-527
  9. McCormack M et al (2015) Fournier's gangrene: A retrospective analysis of 26 cases in a Canadian hospital and literature review. Can Urol Assoc J 9:E407-410
  10. Maguina P et al (2003) Split thickness skin grafting for recreation of the scrotum following Fournier's gangrene. Burns 29: 857-862
  11. Merino E (2002) Fournier's gangrene in HIV-infected patients. Eur J Clin Microbiol Infect Dis 20: 910-913
  12. Morpurgo E, Galandiuk S (2002) Fournier's gangrene. Surg Clin North Am 82: 1213-1224
  13. Nisbet AA, Thompson IM (2002) Impact of diabetes mellitus on the presentation and outcomes of Fournier's gangrene. Urology 60: 775-779
  14. Rouzrokh M et al (2014) Fournier's Gangrene in Children: Report on 7 Cases and Review of Literature. Iran J Pediatr 24:660-661
  15. Xeropotamos NS et al (2002) Fournier's gangrene: diagnostic approach and therapeutic challenge. Eur J Surg 168: 91-95
  16. Yılmazlar Tet al. (2014) Fournier's gangrene: review of 120 patients and predictors of mortality. Ulus Travma Acil Cerrahi Derg 20:333-337

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Last updated on: 29.10.2020