Introduction

Once known as the "strangling angel of children," diphtheria is a preventable, acute, toxin-mediated disease caused by Corynebacterium diphtheriae (Table 1). In the fifth century BCE, Hippocrates described a disease characterized by sore throat, membrane formation, and death through suffocation. In 1826, the French physician Bretonneau named the condition "diphtherite," from the Greek word for leather, because the characteristic membrane resembled leather (1). The bacterium was identified by Klebs in 1884 and first cultivated by Loeffler one year later. Subsequently, Roux and Yersin purified the toxin in 1889, and an antitoxin was invented shortly thereafter, with development of the toxoid in the 1920s (2).

The bacterial exotoxin causes many of the severe manifestations of diphtheria. Clinically, the disease is characterized by pharyngitis and a membrane that may cover the tonsils, pharynx, and larynx. Although diphtheria was one of the most common causes of death of children in the prevaccine era, today diphtheria is rare and seldom considered within the differential diagnosis in developed areas of the world. Sporadic cases do occur, however, and epidemic diphtheria spread through the Soviet Union as recently as the 1990s. Furthermore, the disease is prevalent in many developing countries, and the importation of cases into the United States or other developed countries may occur. The majority of nasopharyngeal C. diphtheriae infections results in asymptomatic carriers, and approximately one in seven individuals develops clinical disease.

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