Clinical Features

TSS should be considered in any unexplained febrile illness associated with erythroderma, hypotension, and diffuse organ pathology. Diagnostic criteria for TSS are listed in T§b!e...l38:l. Patients with MRTSS usually present between the third and fifth day of menses. The median time to onset of illness in postsurgical NMTSS is two postoperative days. There appears to be a spectrum of severity of TSS. Mild cases of TSS may be excluded from the CDC case definition. Mild TSS is generally characterized by fever and chills, myalgias, abdominal pain, sore throat, nausea, vomiting, and diarrhea. Hypotension is usually not present, and the illness is self-limited. Severe TSS is an acute-onset, multisystem disease with symptoms, signs, and laboratory abnormalities reflecting multiple-organ involvement. Headache is the most common complaint. Some patients may experience a prodrome consisting of malaise, myalgias, headache, nausea, vomiting, and diarrhea. Sudden onset of fever and chills occurs approximately 1 to 4 days prior to presentation. Diffuse myalgias, particularly in the proximal aspects of the extremities, abdomen, and back, are reported by virtually all patients; arthralgias are also common. Profuse, watery diarrhea and repeated vomiting are reported by 90 to 98 percent of patients. Orthostatic lightheadedness or syncope may be present. Patients also complain of sore throat, headache, paresthesias, and photophobia. The patient may complain of abdominal pain, cough, or sore throat.

Physical examination reveals hypotension or an orthostatic decrease in systolic pressure of 15 mmHg in all cases. In general, victims of TSS appear acutely ill. The initial state usually lasts about 24 to 48 h; the patient may be obtunded, disoriented, oliguric, and hypotensive. There is an overall body-fluid deficit due to losses from fever, vomiting, diarrhea, and decreased systemic vascular resistance. Depressed cardiac function may also be present. Patients may show nonpitting edema of the face and extremities secondary to nonhydrostatic leakage of intravascular fluid into the interstitium. Other prominent signs may include profound muscle weakness and tenderness or abdominal tenderness. The diarrhea is usually watery and profuse, frequently with associated incontinence. One-half to three-quarters of patients have pharyngitis, with a strawberry-red tongue; conjunctival hyperemia and vaginitis are also seen. Tender, edematous external genitalia, diffuse vaginal hyperemia, "strawberry" cervix, scant purulent cervical discharge, and bilateral adnexal tenderness are seen in 25 to 35 percent of patients with menstruation-related TSS.

The rash of TSS is a diffuse, blanching erythroderma, classically described as painless "sunburn," which fades within 3 days of its appearance and is followed by full-thickness desquamation, especially of the palms and soles, during convalescence. This CDC criterion is most often missed, since it may be subtle or difficult to detect in darkly pigmented patients. Variations include patchy erythroderma and localized maculopustular eruptions. In all cases, a fine, generalized desquamation of the skin, with peeling over the soles, fingers, toes, and palms, occurs from 6 to 14 days after the onset of illness. Most severely ill patients experience loss of hair and nails 2 to 3 months later.

Specific focal neurologic findings rarely occur. Patients present with varying degrees of altered consciousness. Approximately 75 percent of patients have nonfocal neurologic abnormalities without signs of meningeal irritation. Confusion, disorientation, agitation, hysteria, somnolence, and seizures have been reported, consistent with a toxic encephalopathy from cerebral edema. If the clinical picture is unclear, a computed tomography scan and lumbar puncture should be performed. Figure

138-2 illustrates the temporal relationships of the major manifestations of TSS.

FIG. 138-2. Composite drawing of major systemic, skin, and mucous membrane manifestations of toxic shock syndrome. (From Chesney PJ, David JP, Purdy WK, et al: Clinical manifestations of toxic shock syndrome. JAMA 246:741, 1981, with permission.)

Abnormal laboratory values reflect the multisystem involvement in TSS. Leukocytosis, with an increase in immature forms, is frequently seen; lymphocytopenia has also been reported. A mild anemia with acute hypoferrinemia and abnormal peripheral smears consistent with microangiopathic hemolytic anemia or disseminated intravascular coagulation may be found. Azotemia, myoglobinuria, and abnormal urinary sediment (sterile pyuria and red blood cell casts) are seen as acute renal failure develops. Liver function abnormalities and hyperbilirubinemia are seen in approximately 3 percent of patients with clinical evidence of coagulopathy. Metabolic acidosis secondary to hypotension is also seen. Electrolyte abnormalities, including hypocalcemia, hypophosphatemia, hyponatremia, and hypokalemia, are common. Hypocalcemia is out of proportion to the degree of hypoalbuminemia and may be difficult to correct if there is a concomitant decrease in the serum magnesium level.

Acute renal failure secondary to acute tubular necrosis is a complication of TSS. It appears to be secondary to prerenal deficits, renal ischemia caused by hypotension, rhabdomyolysis, and possibly direct damage from TSST-1 mediators. Ventricular arrhythmias, bundle branch block, first-degree heart block, and T-wave and ST-T-wave changes have been reported. Echocardiography of patients with TSS shows wall motion abnormalities and decreased shortening fraction, suggestive of toxic cardiomyopathy. Adult respiratory distress syndrome (ARDS) with refractory hypotension represents the ultimate end-organ damage secondary to TSS. Diagnosis

Other systemic illnesses characterized by fever, rash, diarrhea, myalgias, and multisystem involvement resemble TSS ( Tâb!e..J.38-2). Kawasaki disease (mucocutaneous lymph node syndrome) is characterized by fever, conjunctival hyperemia, and erythema of the mucous membranes with desquamation. Although the exanthems may be quite similar. Kawasaki disease may present with target lesions resembling erythema multiforme, and the bright-red appearance of the vermillion border is not common in TSS. Furthermore, over 80 percent of those afflicted with Kawasaki disease are under 5 years of age, and Kawasaki disease is not characterized by hypotension, renal failure, or thrombocytopenia.

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