Fournier's disease, Fourniers disease, Fournier's gangrene, Fourniers gangrene, Fournier's necrotizing fasciitis of the perineum and genitalia synergistic necrotizing fasciitis of the perineum and genitalia, gangrene, Fournier's necrotising fasciitis of the perineum and genitalia synergistic necrotizing fasciitis of the perineum and genitalia
Authors: Dr Amritpreet Singh, Advanced Trainee in General Medicine, Wellington Regional Hospital, New Zealand; A/Prof Amanda Oakley, Dermatologist, Waikato District Health Board, Hamilton, New Zealand. Copy edited by Gus Mitchell. March 2022
Fournier gangrene is a polymicrobial, rapidly progressivenecrotising fasciitis of the external genitalia, perineum, and perianal region.
It is also known as Fournier disease and necrotising fasciitis of the perineum and genitalia.
Fournier gangrene may be subcategorised by the site of infection:
Fournier gangrene of the penis
Fournier gangrene of the scrotum or perineum
Fournier gangrene of the vagina
Fournier gangrene of the vulva or perineum.
Fournier gangrene is a surgical emergency associated with septic shock, which requires prompt surgical excision and broad-spectrum intravenous antibiotics.
Who gets Fournier gangrene?
Fournier gangrene is rare, with an incidence of 1.6 in 100,000 males. The male to female ratio is 10:1. The lower incidence in females may reflect better drainage of the perineal region.
The typical patient with Fournier gangrene is a male aged 50–79 with comorbid conditions that compromise cellular immunity. These comorbid conditions include:
The risk of contracting Fournier gangrene may be increased by the use of sodium-glucose cotransporter 2 (SGLT2) inhibitors such as empagliflozin for the treatment of type 2 diabetes. These inhibitors can promote the growth of baselineurogenital flora and the risk of localised infection.
What causes Fournier gangrene?
Fournier gangrene originates from a local urogenital or anorectal infection, such as:
Urinary tract infection, epididymitis, or orchitis
Bacteria gain entry into the superficial and deep perineal fascia through trauma, such as:
A genital piercing
Urethral instrumentation or injury
A prosthetic penile implant
A rectal foreign body
Post-coital trauma
Colorectal trauma during surgery.
Infection or trauma is identifiable in 95% of cases of Fournier gangrene.
Once the infection is introduced to the fascia, bacterialtoxins are released. These promote thrombosis of arterioles, causing necrosis of the fascia and localised ischaemia. The resultant tissue hypoxia promotes the growth of anaerobicorganisms, which produce gas and enzymes leading to further necrosis of the fascia.
Fournier gangrene is polymicrobial in 80% of cases, with an average of four microorganisms per infection, including:
Fournier gangrene should be suspected if fluctuance, soft tissue crepitation, localised tenderness of wound are detected on examination of the genitalia and perineum. It is confirmed if gangrenous tissue or pus is found. Examination under anaesthesia may be needed.
Thrombosis of arterioles supplying the superficial and deep fascia is histologicallypathognomonic of Fournier gangrene.
Computed tomography (CT scan) defines the extent of disease and reveals fascial thickening, fat stranding, and soft tissue gas collections.
What is the differential diagnosis for Fournier gangrene?
The differential diagnosis of Fournier gangrene includes:
Fournier gangrene is a surgical emergency. Immediate surgical intervention is imperative if gas is detected clinically or on a CT scan.
Surgical debridement and excision
All necrotic tissue must be removed promptly.
Repeated procedures may be necessary.
Once the infection has been eradicated and healthy granulation tissue has developed, the defect should be reconstructed by primary closure, local skin flap, or a split-thickness skin graft.
Antibiotics
A combination of broad-spectrum intravenous antibiotics is required. For example:
Tazocin and clindamycin
Tazocin and metronidazole
Vancomycin instead of tazocin if methicillin-resistant Staphylococcus aureus (MRSA) is detected
Meropenem instead of tazocin if the patient is allergic to penicillin.
Consider hyperbaric oxygen therapy for clostridial involvement, myonecrosis, or failure of conventional treatment.
General measures
Avoid nonsteroidal anti-inflammatory drugs.
Address underlying comorbidities.
Encourage perineal hygiene.
Avoid urethral and rectal trauma or foreign body.
What is the outcome for Fournier gangrene?
Fournier gangrene is life-threatening and fatal without appropriate treatment.
Diagnosis is often delayed due to the insidious onset of symptoms. The mortality rate is 20–40%. The most common cause of death is septic shock.
Patients often require repeated surgical excisions and remain hospitalised for weeks to months. The minimum time from surgical excision to reconstruction is approximately three weeks.
Following reconstruction, the prognosis of Fournier gangrene is good. However, 50% of men with penile involvement report ongoing pain with erections. If extensive soft tissue is lost, lymphatic drainage may be impaired, resulting in oedema and recurrent cellulitis.
Auerbach J, Bornstein K, Ramzy M, Cabrera J, Montrief T, Long B. Fournier Gangrene in the Emergency Department: Diagnostic Dilemmas, Treatments and Current Perspectives. Open Access Emerg Med. 2020; 12:353–364. DOI: 10.2147/OAEM.S238699. Journal
Bersoff-Matcha S, Chamberlain C, Cao C, Kortpeter C, Chong W. Fournier Gangrene Associated with SGLT Inhibitors: A Review of Spontaneous Postmarketing Cases. Ann. Intern. Med. 2019; 170(11):764–769. DOI: 10.7326/M19–0085. Journal
El-Qushayri AE, Khalaf KM, Dahy A, et al. Fournier’s gangrene mortality: a 17-year systematic review and meta-analysis. Int. J. Infect. Dis. 2020; 92:218–225. DOI: 10.1016/j.ijid.2019.12.030. Journal
Kumar S, Costello AJ, Colman PG. Fournier's gangrene in a man on empagliflozin for treatment of Type 2 diabetes. Diabet Med. 2017; 34(11):1646–1648. DOI: 10.1111/dme.13508. Journal
Pernetti R, Palmieri F, Sagrini E, et al. Fournier's gangrene: Clinical case and review of the literature. Arch Ital Urol Androl. 2016;88(3):237–8. Published 2016 Oct 5. doi:10.4081/aiua.2016.3.237. Journal