Fournier Gangrene: A Rare Flesh-Eating Disease

Described first by Jean-Alfred Fournier in 1883 as an idiopathic condition, Fournier gangrene (FG) is now widely understood to be a fulminant necrotizing fasciitis of the genitals, perineum, and perianal regions.^1-3 FG is a rare and potentially fatal form of necrotizing fasciitis requiring rapid recognition and early surgical intervention.^1,2,4-8 Necrotizing soft tissue infections can progress rapidly and can affect any or all the layers of soft tissue from the dermis through the fascial and muscle compartments.⁸  Failing to promptly identify FG is regarded as one of the greatest impediments to management, as this process can possess an array of early nonspecific symptoms such as tachycardia, erythema, and focal tenderness.^2,4,8 However, as the disease progresses, findings may include pain disproportionate to appearance, hemorrhagic bullae, tense edema, and crepitus in the perineum.⁸ Due in large part to the ambiguous nature of this spectrum of disease progression, a high index of suspicion is required for diagnosis and begins with knowledge of high-risk patient populations.⁸

Population

FG most commonly affects patients with complex comorbidities, such as diabetes mellitus, chronic substance abuse (eg, alcohol, tobacco, and/or intravenous [IV] drug use), obesity, hypertension, or immunosuppression (Table 1).^1,6,7,9,10 Although most often presenting in men in the fifth and sixth decades of life, FG it is not a gender- or age-specific disease.^4,6 In fact, emerging research has concluded that when women develop FG, the clinical course is often protracted and more critical than in men. As a result, it can manifest as prolonged hospital stays, increased ventilator times, and dependence on dialysis during expanded recovery.^3,6

Table 1. Risk Factors for Fournier Gangrene^1,7,9,15

Advanced age

Alcohol abuse

Chronic steroid use

Cytotoxic drugs

Defective phagocytosis

Diabetes mellitus

Diabetic neuropathy

HIV

Hypertension

Lymphoproliferative diseases

Malignancy

Malnutrition

Obesity (BMI >30 kg/m2)

Poor hygiene

Tobacco use

Vascular compromise

Etiology

Generally arising from the gastrointestinal, genitourinary, and integumentary systems, the spectrum of FG causes is vast, ranging from idiopathic to iatrogenic surgical events.^{1,2,7-9,11-13} Of the etiologies implicated, those frequently cited include: appendicitis, diverticulitis, colorectal cancer, complicated nephrolithiasis and its sequelae, and urogenital and perineal abscesses.^9,11,14 Trauma from indwelling catheter placement, rectal or prostate biopsies, diathermy for genital warts, constriction rings for erectile dysfunction, coital injury, genital trauma, hemorrhoidectomy, and hysterectomies have also been implicated.^9,11,14  Of note, the clinical course of FG is dictated not only by host immune defense and the route of opportunistic infection but also by the organism involved.⁸

From the June 01, 2019 Issue of Clinical Advisor