Fournier gangrene N49.8

Baurienne, 1763; Fournier, 1883

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, proctosurgical or gynecological (intensive care) surgical intervention.

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.

• 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.

Perianal 40%; genital region: 40%;

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.

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.

Cytomegaly - Ulcer

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.

High-dose parenteral therapy with broad-spectrum antibiotics such as ampicillin (e.g., Binotal). Adults: 150-200 mg/kg bw/day, children > 1 year of age 100-400 mg/kg bw/day distributed over 4 ED, infants < 1 week of age 50-150 mg/kg bw/day distributed over 2-3 ED, infants > 1 week of age 100-300 mg/kg bw/day distributed over 3-4 ED.

Alternative: benzylpenicillin (e.g., penicillin Grünenthal), adults: 6 times 4 million IU/day i.v., infants > 1st week of life: 50,000-250,000 IU/kg bw/day distributed to 4-6 ED, infants < 1st week of life: 50,000-250,000 IU/kg bw/day distributed to 2 ED, infants > 1st week of life 75,000-350,000 IU/kg bw/day distributed to 4 ED.

Alternative: Gentamicin (e.g. Refobacin) Adults: 3-5 mg/kg bw/day distributed to 1-3 ED, infants > 1st year of life: 5-7 mg/kg bw/day distributed to 3-4 ED, infants < 1st week of life: 5 mg/kg bw/day distributed to 2 ED, infants > 1st week of life: 7.5 mg/kg bw/day distributed to 3 ED combined.

In case of penicillin resistance: 3rd generation cephalosporins, e.g., cefotaxime (Claforan) 3 times/day 2 g i.v.

In therapy resistance: 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 times/day 1 g i.v. with Gentamicin (see above).

The lethality rate is 20-30%.

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