Fournier gangrene N49.8

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%;

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.

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).

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3. Ekelius L et al (2004) Fournier's gangrene after genital piercing. Scand J Infect Dis 36:610-612.
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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
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