Smallpox or smallpox is an extreme and highly contagious infectious disease. It is caused by viruses and transmitted by droplet infection or dust or direct contact. Typical signs are the infectious and contagious pus blisters or pustules. Smallpox, which is often fatal, should not be confused with the more harmless chickenpox in children.
What is smallpox?
Smallpox (variola) is a highly contagious, acute, notifiable infectious disease and should not be confused with the childhood disease of chickenpox. The causative agent is the variola virus, which belongs to the family Poxviridae. Transmission occurs by droplet, smear, and dust infection. The incubation period is 7-11 days. In 1980, the World Health Organization declared this disease eradicated after a worldwide vaccination program. Universal vaccination requirements were lifted; however, international vaccination and quarantine regulations remain in effect. True smallpox (variola major, variola vera) is caused by orthopoxvirus variola. Smallpox has been known as an endemic disease since about 1000 BC in China, India, and Arabia. In Europe, the first reliably documented epidemic occurred in the 6th century, followed by others in England in the 13th century and in Germany at the end of the 15th century. Smallpox was still endemic in Europe in the 19th century; 175,000 cases with more than 100,000 deaths were recorded in Germany in 1871-73. Although smallpox is now thought to have been successfully eradicated, medical science can never be 100% certain that the disease will not break out again someday or somewhere.
Causes
The poxviruses (Poxviridae) are a family of very large, complexly built viruses with an oval or cuboid shape that are widely used as pathogens in vertebrates and insects. With dimensions of 300 × 200 × 150 nm, poxviruses are already recognizable by light microscopy. Infection manifests on skin and mucosa. The genus Orthopoxviruses includes eight interrelated viral strains that lead to infections with skin manifestations and can usually infect only the respective host and closely related host species. In addition to camelid, cow, mouse, and monkeypox viruses, the variola and vaccinia viruses are found here. Variola virus is the causative agent of smallpox in humans, which has been feared for centuries. It is classified in Category A, the highest threat class of bioterror agents, along with the hemorrhagic fever-causing viruses (Ebola and Lassa viruses), anthrax, plague, tularemia (rabbit plague), and botulinum toxin.
Symptoms, complaints, and signs
Probably the most typical and noticeable symptoms of smallpox disease are the papules, which to unsuspecting laypersons are perceived as blisters or rashes. These said papules appear in a wide variety of shapes and sizes. But before such a smallpox rash becomes visible, there are other symptoms and signs. These include, for example, severe back pain, fever, inflammation of the respiratory tract, pain in the limbs or even a long-lasting headache. In addition, there are small red blisters on the tongue and throat, which are considered harbingers of the said smallpox. In most cases, smallpox occurs directly on the face and is associated with severe itching. The constant scratching of the face can even result in open sores, so that inflammation including the formation of pus is possible. After some time, the papules turn into hard crusts, which will then fall off all by themselves. What remains are unsightly scars that persist for some time. Smallpox is accompanied by very distinct symptoms, which are usually associated with severe itching. Only with the right medication can the symptoms that occur be alleviated. Otherwise, a significant aggravation is to be expected.
Course of the disease
After an initial stage of 2-4 days with high fever, headache, lower back pain and pain in the limbs, inflammation of the upper respiratory tract and a temporary rash, the subsequent eruption stage, with a brief drop in fever, results in the characteristic formation of pale red, itchy patches that develop into nodules, vesicles, papules and pustules. They usually spread from the head over the entire body and dry up with bark and scab formation after a few weeks. After rejection of the (infectious) crusts, the typical pockmarks remain.
Complications
Smallpox brings several complications, depending on the intensity of the disease and any treatment of symptoms. The disease of true smallpox leads to death in about 30 percent of cases. In addition, the disease leaves scars in survivors. If the eyes, inner ear, nervous system or other major sensory organs have been severely affected, it often results in blindness, deafness, paralysis or general nerve and brain damage. Smallpox leaves late effects in all affected individuals. A particular complication is the occurrence of hemorrhagic smallpox (black smallpox). Their symptoms are more serious than those of other smallpox diseases. They mean the occurrence of internal bleeding, massive bruising and thus damage to vital organs. Affected persons usually die within 48 hours, or after a few days at the latest. Monkeypox and cowpox are special cases, of which there have been a few in recent years. This mild form of the disease usually heals on its own within a few weeks. However, since it attacks the nervous system, it can also lead to the complications of true smallpox. This is especially true for people with a weakened immune system.
When should you see a doctor?
Smallpox must always be examined and treated by a doctor. If treatment is not received for this disease, the affected person may die from smallpox in the worst case. In most cases, the symptoms intensify over time and there is no self-healing. A doctor should be consulted for smallpox if there is a severe rash on the skin of the affected person and the formation of papules. This is often accompanied by severe back pain, fever or even pain in the limbs and headache. Therefore, if these symptoms occur along with the papules, a doctor should be consulted immediately. In some cases, smallpox also manifests itself through respiratory illness. The disease can be diagnosed and treated by a general practitioner or in a hospital. With the help of protective vaccinations, a fatal course of smallpox can be avoided. Early treatment by a physician can also prevent a fatal course.
Treatment and therapy
The actual vaccine protection against smallpox was made possible in 1796 by E. Jenner through vaccination with bovine smallpox lymph. He thus laid the foundation for successful control in conjunction with later vaccination laws. If smallpox were to be used as a biological weapon, it would pose a serious threat to civilians. Mortality is 30% (or more) in unvaccinated individuals, and furthermore, there is no specific treatment option. Although smallpox was the most feared infectious disease for a long period of time, its potential threat is greater today than in previous centuries. Mandatory vaccination was discontinued in many countries about 25 years ago. Vaccination given within four days of exposure appears to give some protection against smallpox infection and significant protection against a fatal course. Smallpox vaccine is now available worldwide as a contingency countermeasure. Vaccinia and other smallpox viruses are used in the life sciences as vectors for the expression of foreign proteins, and also for the development of novel vaccines. Survival of the disease confers lifelong immunity. In case of incomplete immunity, the course is strongly attenuated in the 2nd phase (variolois). A milder form of the disease is white pox or milk pox (variola minor or alastrim), which is caused by orthopoxvirus alastrim; it leaves no immunity to true smallpox.
Follow-up
Smallpox is widely considered eradicated. Patients who have contracted smallpox and overcome the condition must continue to seek medical care. After completion of therapy, physicians check the patient’s state of health at regular intervals. In this way, accompanying symptoms can be detected at an early stage. Despite comprehensive treatment, the viruses may have survived in the body and lead to smallpox disease again. Since the symptoms usually return in the first few weeks to months, follow-up care takes place much more frequently at the beginning. The appointments can be gradually reduced.Accompanying this, rest and bed rest apply because the condition has a strong effect on the immune system. As a precautionary measure, the skin should continue to be spared and any injuries or scarring treated according to the doctor’s instructions. Follow-up care is carried out by a dermatologist in private practice, who contacts the family doctor in charge as well as the internists involved. For this purpose, the physician requires all important documents and medical records. In the course of follow-up care, further screenings may be performed in order to exclude permanent damage to the skin and secondary diseases or to detect abnormalities in time. Further follow-up measures always depend on the course of the disease.
What you can do yourself
Since smallpox is one of the diseases that are highly contagious and also fatal in an unfavorable course, affected persons should consult a doctor at the first signs and be sure to follow the doctor’s instructions. The disease is considered eradicated in our region. Therefore, there are hardly any indications for self-help. Nevertheless, if irregularities occur, there is a special duty to act. Because of its explosive nature, smallpox is notifiable and is subject to international quarantine regulations. Independent health care is neither recommended nor permitted. As protection against the disease, timely vaccination is recommended. This should be done on one’s own responsibility, so that immunity to the pathogens occurs. If a case of the disease becomes known in the immediate vicinity, consultation with a physician should also be sought as soon as possible,
The disease is associated with symptoms such as high fever. Although a loss of appetite and strength occurs, care must be taken to ensure that sufficient nutrients are available to the organism. The amount of fluids must be adapted to the needs of the body and must be increased compared to normal circumstances. The changes in the appearance of the skin cause intense itching. Despite the discomfort, care should be taken not to give in to the itching sensation if possible. This increases the risk of sepsis, which should be prevented.