123 Scarlet Fever
Scarlet fever is an acute infectious disease, caused by certain types of group A hemolytic streptococci. The disease is characterized by sudden onset of soreness on swallowing, with fever and headache.
A rash appears within 2 days of onset, and desquamation follows.The term “scarlet fever” was supposedly first used by Thomas Sydenham in 1683, but it appeared in a diary of Samuel Pepys in an entry for November 10, 1664. From the seventeenth to the early twentieth century, the word scarlatina was popularly used to denote a mild form of the disease.
Distribution and Incidence
Like the streptococcal sore throat to which it is closely related, scarlet fever is a disease of temperate climates, prevailing generally in the winter months. It occurs principally in young children, although adults may suffer sore throats as a result of the same infection.
Etiology and Epidemiology
The group A hemolytic streptococci are responsible for a range of afflictions other than scarlet fever, including erysipelas, rheumatic fever, and the sore throats known as tonsillitis in Great Britain and as pharyngitis in the United States. Scarlet fever is caused only by certain strains that produce (or release) a soluble toxin, whose absorption causes the rash characteristic of the disease. Different strains of streptococci produce different amounts of toxin. Epidemics thus vary greatly in severity, with mortality rates ranging from 0 to 30 percent. Transmission of the infection is by intimate contact, such as occurs in overcrowded homes and classrooms, and evidence of airborne or droplet nuclei infection is slight. In the past, scarlet fever occasionally occurred as a hospital infection, and the disease was also transmitted in contaminated milk.
Susceptibility to the skin rash differs according to the immune and hypersensitivity status of the individual.
Those who have experienced scarlet fever once are unlikely to do so again, but remain vulnerable to streptococcal sore throats when exposed to infection with a new serologic type. Research on the susceptibility of different population groups, as defined by a positive skin test (Dick test), suggests that more than half of young infants are immune to the disease, but that by the age of 2 years, only some 20 percent remain so. Thereafter the proportion of im- munes rises steadily through childhood, reaching 77 percent at 10 to 15 years and 86 percent in adults.The rare occurrence of second attacks of scarlet fever with rash is probably due to infection with a new antigenic erythrogenic toxin. The available evidence suggests that the geographic dominance of particular strains of scarlet fever streptococci is long-term, varying from country to country and from time to time. In Britain, during the years 1936 to 1956, type 4 streptococci were isolated more often from scarlet fever than from tonsillitis cases. In 1964-5, the commonest type associated with scarlet fever was type 4 in Britain, type 22 in East Germany, and type 1 in the former Soviet Union and Holland. With all types, the disease appears to follow a general pattern of alternate severity and mildness. At present, it is very common, though very mild, in both Europe and North America. Fatalities have ceased to occur, and the prevailing mildness of type means that cases tend to escape notification.
Clinical Manifestations
The initial symptoms of scarlet fever are the same as those of streptococcal sore throat: sudden onset of soreness on swallowing, accompanied by fever and headache. Vomiting and nausea are often early symptoms in young children. The characteristically erythemateous and punctuate rash appears within 2 days, at first on the upper chest and back, then spreading to the rest of the body. In white patients the rash does not commonly appear on the face, but in about half of all black patients it does.
The rash is accompanied by the characteristic raspberry tongue. In general, the rash is variable in its manifestations. Desquamation usually occurs, beginning sometimes as early as the fifth day, sometimes as late as 4 to 5 weeks after the onset of the disease. A range of complications, principally affecting young children, add to the dangers of the disease. These include anemia, otitis media, rheumatic fever, and meningitis. In rare cases, scarlet fever appears in severe septic or toxic forms.History and Geography
The early history of scarlet fever prevalence is obscure. It is possible that outbreaks were observed by Near Eastern practitioners of the Arabian school, but the first undoubted account of a disease with a fiery rash as a characteristic was provided by Giovanni Filippo Ingrassia of Palermo in 1553. The disease was apparently present in Germany and Italy in the early seventeenth century, and we know of a severe outbreak in Poland in 1625. At that time, the disease was variously known as rossalia, purpurea epidemica maligna, and febris miliaria rubra. It is clear from observations by Daniel Sennert in 1619, Michael During in 1625, and Johann Schultes in 1665 that the scarlatinal manifestations of desquamation, nephritis, and dropsy were well known before the disease received its modern name. Although in 1683 Sydenham wrote of the disease as having a mild character, he nevertheless established its autonomy, and distinguished it from other acute exanthema, notably measles, by naming it. By the end of the seventeenth century, the identity of scarlet fever was well recognized, although much epidemiological confusion remained, and still remains, over the respective roles of scarlet fever, streptococcal sore throat, and diphtheria (cynanche maligna) in seventeenth-century and eighteenthcentury epidemics.
During the eighteenth century, scarlet fever was present in epidemic form throughout Europe and the United States. It appeared in Copenhagen in 1677, in Scotland in 1684, in the United States in 1735, and in Sweden in 1744.
In general, however, the evidence suggests that the disease made irregular epidemic appearances, and that its mortality varied considerably. During the early eighteenth century, it seems to have been of fairly mild character, but Dutch and Swedish evidence suggests that by midcentury a very virulent strain was also present.The character of scarlet fever as a relatively new disease may be reflected in the age incidences reported during this period. Sydenham noted that it attacked whole families, though more especially the infants, whereas Nils R6sen von Rosenstein observed in 1744 the simultaneous occurrence of sore throat without rash in children in infected households. This pattern was observed in adults by Maxmilian Stoll in 1786. In the last years of the century, scarlet fever was extensive and virulent in Europe, with severe outbreaks in Denmark and Finland in 1776-8, and in central Germany in 17951805. By 1814 it was again very mild, but continued its global spread, appearing in South America in 1892, in Greenland in 1847, and in Australia and New Zealand in 1848.
During the 1820s and 1830s, however, a more virulent form reappeared, and consequently, the disease was the leading cause of death among infectious childhood maladies until 1875. During the 1880s, the disease continued to be widely prevalent but began to decline as a cause of death, and by the 1890s its character was again relatively mild, although not as mild as it has become today. This decline in severity was first apparent in Britain and western Europe, although a malignant form was still present in Poland, Russia, and Romania during the 1930s. Observations by Edward Wilberforce Goodall made in the Metropolitan Asylums’ Board’s Eastern Hospital, London, showed that as fatality dwindled, so did the more serious clinical forms.
Streptococci were first isolated from the blood of scarlet fever patients by Edward Klein in 1887, but he failed to reproduce the disease in animals. In 1911 Kari Landsteiner produced a similar disease in monkeys by inoculating them with faucial exudate from scarlet fever patients, but until about 1922 the streptococci were generally considered to be secondary invaders.
It was ultimately observation of human beings that proved scarlet fever to be a result of streptococcal infection. Seminal work by George Dick and Gladys Dick in the early 1920s proved scarlet fever to be primarily a local infection of the throat caused by type A hemolytic streptococci. In 1923 the Dicks successfully inoculated volunteers, and in 1924 developed the Dick test: the intradermal injection of a diluted filtrate of a broth culture of a scarlatinal strain of streptococcus which, by the resultant appearance or not of a local erythemateous reaction', determines the susceptibility of the subject to scarlet fever. In other words, a negative Dick test is an indication of antitoxic immunity.
Anne Hardy
Bibliography
Rolleston, J. D. 1937. The history of acute exanthema. London.
Smith, Francis B. 1979. The people’s health, 1830—1910. London.
Wilson, L. G. 1986. The historical riddle of milk-bome scarlet fever. Bulletin of the History of Medicine 60: 321-42.