J.T. is a 43-year-old man who presented to his primary care doctor after 4 days of progressive pain and swelling in his genital region, along with a low-grade fever. There was no history of similar symptoms. He denied recent trauma. He had no nausea, vomiting, diarrhea, constipation, abdominal pain, melena, or hematochezia. He also denied dysuria, urgency, and frequency. He was in a monogamous relationship and had no history of sexually transmitted diseases. Notably, he reported a 110-lb weight loss over 6 years.
His medical history was positive only for a urethral stricture treated with a urethral dilation on cystoscopy in 1965. He had no allergies and was taking no medications. He did not smoke or drink alcohol. His family history was significant for his grandmother with type 2 diabetes.
Physical examination showed a mildly ill-appearing man in moderate distress. He was awake and alert. His vital signs included a blood pressure of 115/72 mmHg, heart rate of 110, respiratory rate of 13, and temperature of 99.7 degrees. He was 5′9″ and weighed 140 lb. Head, eyes, ears, nose, and throat were normal. Lungs were clear. Heart was tachycardic with a normal s1 and s2 with no murmurs or gallops and no rub. Abdomen was nontender and nondistended with normal bowel sounds. His extremities had no edema, and pulses were normal bilaterally.
J.T.’s genital exam revealed diffuse erythema and edema of his scrotum and perineal area, along with severe tenderness. There were multiple areas of hemorrhagic necrosis involving a large part of the scrotum but sparing the penis. His testicles were normal in size and contour and were not tender. His perirectal area was erythematous, but there was no evidence of fissures, ulcerations, or crepitus.
Laboratory data included a normal hemoglobin and hematocrit but a markedly elevated white blood count to 35,000 k/mm3. His chem. 7 panel was also normal except for a mildly decreased sodium of 132 mEq/l and an elevated glucose of 284 mg/dl. His HbA1c concentration was 12.8% with a glycosylated hemoglobin of 17.7%. There were no other laboratory abnormalities. No other laboratory tests or imaging procedures were ordered.
What is the diagnosis?
What are the potential sources?
How is this patient predisposed to developing this condition?
How should this patient be managed?
This is a classic presentation of Fournier’s gangrene, which is defined as necrotizing cellulitis and fascitis of the perineum. Originally called “spontaneous fulminant gangrene of the scrotum,” Fournier’s gangrene was first described by Baurienne1 in 1764 but named after Jean Alfred Fournier2 in 1883, after he characterized the disease as the abrupt onset of fulminating genital gangrene of idiopathic origin in previously healthy young patients.
Since then, our understanding of this disease has changed considerably. Fournier’s gangrene is now considered to have a somewhat more indolent course, to have an identifiable source in 75–100% of cases, to be associated with systemic disease, and to occur throughout a broader age range.3,4 The incidence is higher in men than in women, with a ratio of 10:1.5
Fournier’s gangrene most often affects patients with systemic disease, most commonly diabetes or alcoholism. Studies have shown the prevalence of diabetes in patients with Fournier’s gangrene to be 32–60% and the prevalence of alcoholism to be between 25 and 66%.3,4 Chronic debilitation and immunosuppression secondary to transplantation, chemotherapy, or HIV infection are also significant risk factors.3,4
Patients often present with genital swelling, erythema, and tenderness, frequently accompanied by fever and systemic toxicity. Swelling quickly increases, and crepitus and gangrene develop.
A rectal, dermal, or urinary source is often found by careful investigation.3,4 Rectal sources are suggested by pain, bleeding, and a history of anal fissures or dilatation, hemorrhoid banding, or perforation. Dermal sources are suggested by acute or chronic infections of the scrotum, recurrent hidradenitis suppurativa, balanitis, or recent epesiotomy. Urinary sources are suggested by dysuria, urethral discharge, recent urinary catheterization, or obstructed voiding, especially as a result of urethral strictures from sexually transmitted diseases. Of all the predisposing conditions, urethral strictures and perirectal abscesses are the most common.6
In contrast to historical descriptions, the microbiological etiology can often be determined. The lesions are usually foul smelling, suggesting a polymicrobial infection with anaerobic-aerobic synergy.7 Organisms typically isolated include clostridia, klebsiella, streptococci, coliforms, staphylococci, bacteroides, and corynebacteria.5 Local infection usually occurs next to the portal of entry. The inflammatory reaction then spreads to the deep fascial planes with resultant obliterative endarteritis. This results in a cascade of cutaneous and subcutaneous vascular necrosis, local ischemia and tissue necrosis, and hypoxia-driven proliferation of anaerobic organisms.3
Despite our knowledge of the etiology of Fournier’s gangrene, the diagnosis must remain clinical because it represents a surgical emergency. Often the illness is too fulminant to await the results of diagnostic tests. Skill is required to differentiate Fournier’s gangrene from scrotal cellulitis, abscesses, strangulated herniae, pyoderma ganrenosum, and vasculitis.3 If time permits, an abdominal X-ray can be used to evaluate for subcutaneous air in the abdominal wall. Similarly, a scrotal ultrasound can help to evaluate for subcutaneous air before the clinical detection of crepitus and to rule out other scrotal pathology.3 Proctoscopy can be useful to evaluate for the source of infection and to document rectal and anal involvement.4 Likewise, a retrograde urethrogram can demonstrate urinary extravasation, thus documenting the need for suprapubic diversion.4
Fournier’s gangrene is a surgical emergency. Treatment consists of aggressive, prompt surgical debridement, and intravenous antibiotics and fluids. Most experts recommend treating with a penicillin to cover clostridia, an aminoglycoside or third-generation cephalosporin for gram negative rods, and clindamycin (Cleocin) or metronidazole (Flagyl) for anaerobes.5
A wide debridement should be performed until normal fascia is found and repeated debridement is often required.6 Although occasionally compromised, the testes are frequently spared because of their independent blood supply.3 A colostomy is performed if there is colonic or rectal perforation. Recently, hyperbaric oxygen has been shown to decrease length of stay, increase wound healing, and decrease the spread of infection.8
Mortality is ∼16%5 and has not improved much since the original description of this disease, likely because of the more complicated patient population and the increased average age of patients at diagnosis.7 Interestingly, despite being the highest-risk group for developing Fournier’s gangrene, diabetic patients are not at increased risk of death compared to other patients with the disease. However, mortality is increased with advanced age, extensive disease, shock or sepsis, positive blood cultures, anorectal sources, and renal or hepatic dysfunction.4,5,9
J.T. was diagnosed as having type 2 diabetes and Fournier’s gangrene and was taken to the operating room for debridement within several hours of being transferred to the emergency room from his primary care physician’s office. His testicles were preserved, and he did not require a colostomy. He was hospitalized for 10 days and underwent three surgical debridements and a flap closure of his wound. He remained on ampicillin (Unasyn) until discharge and was then placed on amoxicillin (Augmentin).
He received diabetes education and performed self-monitoring of blood glucose four times a day. He was treated with insulin. His diabetes was then managed as an outpatient with no subsequent readmissions.
Fournier’s gangrene is a rare condition that requires emergency surgical treatment.
The clinical presentation can be variable but may consist of genital swelling, erythema, and tenderness, frequently accompanied by fever. Presentation often occurs before the development of crepitus. The disease may be indolent with progression over several days.
Suspect a systemic disease such as diabetes, alcoholism, or HIV infection in a patient diagnosed with Fournier’s gangrene.
Search for the portal of entry, which typically includes the dermis, rectum, or urethra.
Mortality is still almost 20% despite advances in modern medicine.
David J. Meier, MD, is a house officer in the Department of Internal Medicine at the University of Michigan in Ann Arbor.