Early recognition and treatment of necrotizing fasciitis (NF) is essen
tial for survival. The diagnosis of primary or idiopathic NF may be pa
rticularly challenging because it occurs in the absence of a known cau
sative factor or portal of entry for bacteria. Patients with NF treate
d between 1989 and 1993 were reviewed to determine the incidence, clin
ical features, bacteriology, and results of treatment in patients with
idiopathic NF. Idiopathic NF occurred in nine (18%) of 51 patients, f
ive men and four women, ranging in age from 21 to 67 years. Associated
conditions included diabetes mellitus (4), alcoholism (3), remote inf
ection (3), and pregnancy (2). NF affected the lower extremity in eigh
t and the perineum in one patient. Pain and tenderness occurred in all
patients, soft tissue gas was recognized in two, and the presence of
erythema and edema was variable. Idiopathic NF was monomicrobial in se
ven (78%) patients, compared to 21 per cent of patients with secondary
NF (P = 0.003). S. pyogenes was the causative organism in five of sev
en monomicrobial infections. Time from admission to operation was sign
ificantly longer (62.3 +/- 54.8 hours) in patients with idiopathic NF
compared to patients with secondary NF (17.0 +/- 16.6 hours) (P = 0.00
1). Treatment included operative debridement (xBAR = 3.3) and limb amp
utation (n = 1) to control infection. Three patients (33%) with idiopa
thic NF died. Primary or idiopathic NF is principally a monomicrobial
infection usually caused by S. pyogenes that most commonly occurs in t
he extremities. Mortality is high but is comparable to secondary NF. I
t is important to recognize that NF may occur spontaneously, and it sh
ould be suspected in patients with unexplained soft tissue pain and te
nderness.