Smallpox: Types, Symptoms & Treatments Read This Next What Is Smallpox? Smallpox is an extremely contagious and deadly virus for which there is no known cure. The last known case occurred in the United States in 1949 and due to worldwide vaccination programs, this disease has been completely eradicated. Smallpox is also known as variola. Since the time of ancient Egypt, smallpox has proven to be one of the most devastating diseases to humankind. Widespread smallpox epidemics and huge death tolls fill the pages of our history books. The first smallpox vaccine was created in 1758 . However, the disease continued to infect and kill people on a widespread basis for another 200 years. The World Health Organization (WHO) implemented a strict vaccination standard in order to slow the infection rate. The last known natural case occurred in 1977 in Somalia. By 1980 , the WHO declared that smallpox had been completely eradicated, although government and health agencies still have stashes of smallpox virus for research purposes. People no longer receive routine smallpox vaccinations.

The smallpox vaccine can have potentially fatal side effects, so only the people who are at high risk of exposure get the vaccine. What Are the Symptoms of Smallpox? Historical accounts show that when someone was infected with the smallpox virus, they had no symptoms for between seven and 17 days. However, once the incubation period (or virus development phase) was over, the following flu-like symptoms occurred: high fever abdominal pain vomiting These symptoms would go away within two to three days. Then the patient would feel better. However, just as the patient started to feel better, a rash would appear. The rash started on the face and then spread to the hands, forearms, and the main part of the body. The person would be highly contagious until the rash disappeared. Within two days of appearance, the rash would develop into abscesses that filled with fluid and pus. The abscesses would break open and scab over. The scabs would eventually fall off, leaving pit mark scars. Until the scabs fell off, the person remained contagious. Types of Smallpox There were two common and two rare forms of smallpox. The two common forms were known as variola minor and variola major.

Variola minor was a less fatal type of smallpox. The Centers for Disease Control and Prevention (CDC) estimate that only 1 percent of those infected died. However, it was less common than variola major. The CDC estimates that 90 percent of smallpox cases were variola major. Historically, this type of smallpox killed 30 percent of those infected. The two rare forms of smallpox were known as hemorrhagic and malignant. Both of these rare forms of smallpox carried a very high fatality rate. Hemorrhagic smallpox caused organs to leak blood into the mucous membranes and skin. Malignant smallpox lesions did not develop into pustules or pus-filled bumps on the skin. Instead, they remained soft and flat throughout the entire illness. How Do You Catch Smallpox? One of the reasons smallpox was so dangerous and deadly is because it’s an airborne disease. Airborne diseases tend to spread fast. Coughing, sneezing, or direct contact with any bodily fluids could spread the smallpox virus. In addition, sharing contaminated clothing or bedding could lead to infection. Treatment for Smallpox There is no cure for the smallpox virus.

As a result of worldwide, repeated vaccination programs, the variola virus (smallpox) has been completely eradicated. The only people considered to be at risk for smallpox are researchers who work with it in a laboratory setting. In the unlikely event that an exposure to the smallpox virus occurs, vaccination within one to three days can keep the illness from being so severe. In addition, antibiotics can help to reduce the bacterial infections associated with the virus. Article Resources Article resources Riedel, S. (2005, January). Edward Jenner and the history of smallpox and vaccination. Proceedings of Baylor University Medical Center, 18(1), 21-25. Retrieved from Smallpox. (2014, October 22). Retrieved from Smallpox. (2015). Retrieved from Smallpox disease overview. (2007, February 6). Retrieved from Smallpox fact sheet vaccine overview. (2007, February 7). Retrieved from Smallpox Symptoms, Treatment, Causes - What causes smallpox? - MedicineNet Medical Author: Charles Patrick Davis, MD, PhD Charles Patrick Davis, MD, PhD Dr.

Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications. Medical Editor: Melissa Conrad Stöppler, MD Melissa Conrad Stöppler, MD Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology. Where can people find more information on smallpox? What causes smallpox?

Smallpox is caused by a poxvirus called variola (Poxviridae family of viruses, genus Orthopoxvirus). Variola is a relatively large virus that contains double-stranded DNA. The virus can be found in large numbers in many organs (skin, kidneys, spleen, liver , and other organs) of infected people. Death occurs because of overwhelming toxemia, thought to be due to immune complexes trying to react to the large number of viral particles. Variola infection only occurs in humans, which was helpful in eradicating the disease. There are two strains called variola major and variola minor (also known as alastrim). As implied by the names, variola major is more likely to cause serious disease and death than variola minor. What are the risk factors for smallpox? In the past, the major risk factor for getting smallpox was close association with another person with smallpox who expelled virus-contaminated droplets in coughs and sneezes; infrequently, some patients may have obtained the disease from touching heavily contaminated items and then transferring viruses to their own mucous membranes.

Currently, the major risk factors are working in highly specialized laboratories that may still have smallpox viruses in storage by accident or become contaminated while either working with the viruses or using the viruses as a biological weapon. Medically Reviewed by a Doctor on 9/7/2016 Smallpox: MedlinePlus Medical Encyclopedia URL of this page: //medlineplus.gov/ency/article/001356.htm Smallpox Smallpox is a serious disease that is easily passed from person to person (contagious). It is caused by a virus. Causes Smallpox spreads from one person to another from saliva droplets. It may also be spread from bed sheets and clothing. It is most contagious during the first week of the infection. It may continue to be contagious until the scabs from the rash fall off. The virus can stay alive between 6 and 24 hours. People were once vaccinated against this disease. However, the disease has been mostly wiped out. The United States stopped giving the smallpox vaccine in 1972. In 1980, the World Health Organization (WHO) recommended that all countries stop vaccinating for smallpox.

There are two forms of smallpox: Variola major is a serious illness that can be life threatening in people who have not been vaccinated. It was responsible for a large number of deaths. Variola minor is a milder infection that rarely causes death. A massive program by the WHO wiped out all known smallpox viruses from the world in the 1970s, except for a few samples saved for government research. Researchers continue to debate whether or not to kill the last remaining samples of the virus, or to preserve it in case there may be some future reason to study it. You are more likely to develop smallpox if you: Are a laboratory worker who handles the virus (rare) Are in a location where the virus was released as a biological weapon It is unknown how long past vaccinations stay effective. People who received the vaccine many years ago may no longer be fully protected against the virus. THE RISK OF TERRORISM There is a concern that the smallpox virus could be spread as part of a terrorism attack. The virus could be spread in spray (aerosol) form. Symptoms Symptoms usually occur about 12 to 14 days after you have been infected with the virus.

They may include: Backache Raised pink rash, turns into sores that become crusty on day 8 or 9 Severe headache Infectious Disease Index - Variola virus | MSDSonline PATHOGEN SAFETY DATA SHEET - INFECTIOUS SUBSTANCES SECTION I - INFECTIOUS AGENT NAME: Variola virus(1,2). SYNONYM OR CROSS REFERENCE: Smallpox, VARV, and variola minor virus strains (alastrim, amass, or kaffir viruses)(1,2,3). CHARACTERISTICS: A member of the family Poxviridae , subfamily Chordopoxvirinae , genus Orthopoxvirus . Virions are shaped like bricks on electron micrographs and measure approximately 300 x 250 x 200 nm(2,4). Orthopoxviruses have an outside envelope and a second membrane underneath. Instead of a capsid, poxviruses have a nucleosome which contains DNA, and is surrounded by its own membrane(5). They contain single, linear, double-stranded DNA molecules of 130 to 375 kb pairs and replicate in the cell cytoplasm(4).

Variola virus is the most complex of the orthopoxvirus genus, having many different strains(6,7). SECTION II – HAZARD IDENTIFICATION PATHOGENICITY/TOXICITY: Smallpox is an acute, contagious disease with two main forms, variola major and variola minor, both of which cause similar lesions(1). There are 4 types of variola major infection presentations. Ordinary variola major : The most common form, which accounts for more than 90% of cases and has a fatality rate of approximately 30% among unvaccinated individuals, and 3% for vaccinated individuals(8). The cause of death is usually bronchopneumonia, although in about 3% of cases, fatal haemorrhages occur. The prodromal phase consists of sudden onset of influenza-like symptoms, characterized by fever, malaise, headache, prostration, severe back pain, and sometimes abdominal pain and vomiting. Two to 3 days later, the temperature falls and the patient feels somewhat better, at which time a characteristic rash appears, first on the face (starting as small red spots) then on the tongue, mouth, nose, and hands.

After a few days, the rash progresses to the trunk where fewer lesions occur. Lesions in the mucous membranes of the nose and mouth ulcerate quickly, releasing large amounts of virus into the mouth and throat. Lesions progress from macules to papules to vesicles to pustules, and at 8 to14 days, the pustules form scabs which leave depressed depigmented scars upon healing. All lesions in a given area progress through these stages together(1,8). The milder or "modified" variola major : Accounts for 2% of cases in unvaccinated persons and for 25% in previously vaccinated persons. Cases are rarely fatal with fewer, smaller, and more superficial lesions than those in patients with the ordinary type(4). Haemorrhagic variola major : A rare form of variola major which is always fatal and involves haemorrhages in the mucous membranes and the skin(1,4). Flat variola major : Another rare form of variola major that is almost always fatal and is characterized by lesions that do not develop to the pustular stage, but remain soft and flat(2,4).

Variola minor : The other main form of smallpox (also known as alastrim), which is a milder illness with a fatality rate of less than 1%(1). Another type of smallpox, variola sine eruptione , occurs in previously vaccinated contacts or in infants with maternal antibodies. Affected persons are asymptomatic or have a brief rise in temperature, headache, and present influenza-like symptoms. The transmission of clinical smallpox has not been documented with variola sine eruptione(4). Overall, 65% to 80% of smallpox survivors have pockmarks, mostly on the face(1). EPIDEMIOLOGY: As a result of a successful worldwide vaccination program, smallpox was eradicated, with the last natural infection occurring in Somalia in 1977(1,8). HOST RANGE: Humans(1). Monkeys are also susceptible to infection(8). INFECTIOUS DOSE: Viruses in an aerosol suspension can spread widely, and infect at a very low dose (10 to 100 organisms)(8,9,10).

MODE OF TRANSMISSION: Transmission occurs via respiratory droplets (primary route of transmission), or via fine-particle aerosol, or skin inoculation. The conjunctiva or placenta may be occasional portals of entry(1). Respiratory droplets (i.e., coughing, sputum, and saliva) have a range of likely no more than 2 metres and are, therefore, a threat only to persons in the immediate vicinity of the affected patient(11). INCUBATION PERIOD: Can range from 7-19 days(12). Typically, onset of illness occurs after 10-14 days and 2-4 more days for onset of rash to occur(4,9,12). COMMUNICABILITY: The highest risk of transmission is during the appearance of the earliest lesions; however, the period of communicability is from the development of the earliest lesions to the disappearance of all scabs (about a 3 week period)(1). Some infected people shed the virus without ever showing signs of illness(5). SECTION III - DISSEMINATION RESERVOIR: No known animal or environmental reservoir.

Currently, the virus isolates are maintained only in World Health Organization (WHO) designated laboratories(1). ZOONOSIS: None. VECTORS: None. SECTION IV – STABILITY AND VIABILITY DRUG SUSCEPTIBILITY: The acyclic nucleoside phosphonate analogue cidofovir has been shown to have activity against variola virus in cell culture, and in animal models, including animals where therapy was delayed until the first signs of smallpox(13,14). SUSCEPTIBILITY TO DISINFECTANTS: Inactivation can be achieved using apolar lipophilic solvents (chloroform) and quaternary ammonia compounds(15). Other disinfectants used for standard hospital infection control such as 0.5% or 200 ppm hypochlorite, 40% ethanol, 30% isopropyl alcohol, formalin, formaldehyde, phenol, cresol, surface-active detergents (e.g. 100 ppm benzalkonium chloride), chlorobenzene mixtures, and 75 ppm iodophor may be effective(15,16,17,18,19).

PHYSICAL INACTIVATION: Inactivated by heat (incineration and autoclaving)(18). SURVIVAL OUTSIDE HOST: The virus can be propagated in a monkey kidney cell line(17). Specimens of blood, scrapings from skin lesions, saliva, pustular fluid, and crusts can be transported and stored for short periods without refrigeration. Materials from smallpox patients (dried fluid and crusts) containing virus remain infectious at room temperature for approximately 1 year. The infectivity of the virus is maintained at 4°C for several months and at -20 to -70°C for years(15). Based on the behaviour of vaccinia virus, it is believed that aerosolized variola can retain its infectivity for up to 24 hours if not exposed to UV light, and if temperatures are cool (10°C to 11°C) and humidity low (20%). Variola can be almost completely destroyed within 6 hours in an atmosphere of high temperature (31°C to 33°C) and humidity (80%).

At cooler temperatures (26°C), variola virus has survived for 8 weeks at high relative humidity and 12 weeks at a relative humidity less than 10%. Virus has been isolated from scabs that had been sitting on a shelf for 13 years(9). It was also found to be viable in bread, salad, sausages and gauze bandages stored at 4°C for up to 2 weeks, and in storm water kept at 4.5 °C for up to 166 days(5). Samples of freeze-dried virus in a laboratory have been revived after storage for 20 years(20). SECTION V – FIRST AID / MEDICAL SURVEILLANCE: In the past, smallpox was sometimes confused with chickenpox. The centrifugal distribution of lesions, mostly on the face and extremities than on the trunk, is a distinctive diagnostic feature of smallpox which serves to distinguish it from chickenpox, which is characterized by much more superficial lesions that are concentrated mostly on the trunk, as opposed to the face and extremities(1,8). There are several methods for confirming the diagnosis.

Some are specific for variola virus, and others are for orthopoxviruses in general. Haemadsorption with susceptible chicken erythrocytes is an early detection method for infection with smallpox virus. Giemsa-stained smears of material from skin lesions may show Guarnieri inclusion bodies. The soluble antigens in blood, vesicle fluid, pustule fluid, and saline extracts from crusts or scrapings in certain stages of disease can be detected via complement fixation, haemagglutination inhibition, immunofluorescence, and Ouchterlony techniques. Serologic response is variable in partially immune patients, who may present clinically with variola sine eruptione(15). Specimens such as vesicular or pustular fluids or scabs can be examined directly for the presence of virions by electron microscopy, and viral antigen can be identified by immunohistochemical studies. Isolation of the virus in live-cell cultures, followed by PCR, or growth on chorioallantois, is confirmatory; however, PCR diagnostic techniques are more accurate(4).

The results of serologic testing do not differentiate among orthopoxvirus species, and paired serum samples are required to distinguish recent infection from vaccination in the remote past. Newer methods, that detect IgM responses, may enhance the sensitivity and specificity of serological test(4). Other diagnostic methods such as immunodiffusion technique, ELISA, restriction fragment-length polymorphisms, and in situ hybridization have been suggested by various laboratories for confirming the presence of Variola(1,8,9,21). Note: All diagnostic methods are not necessarily available in all countries. FIRST AID/TREATMENT: If possible, a suspected case of smallpox should be managed in a negative-pressured room, and the patient should be vaccinated, particularly in an early stage of illness. Strict respiratory and contact isolation is imperative. When there are many patients, an isolation hospital or other facility should be designated. Penicillinase-resistance antimicrobial agents should be used if smallpox lesions are secondarily infected, if bacterial infection endangers the eyes, or if the eruption is very dense and widespread. Daily eye rinsing is required in severe cases. Patients need adequate hydration and nutrition.

Topical idoxuridine should be considered for the treatment of corneal lesions, although its efficacy is unproved for smallpox(4,15). Leukocyte transfer from immuno-compromised persons, and methisazone have been used to treat Vaccinia gangrenosa (or progressive vaccinia), which is characterized by a slowly progressive enlargement and necrosis or gangrene of the skin at the site of smallpox vaccination that can be fatal if treated with non-specific measures only (antibiotics or steroids alone)(22). IMMUNIZATION: The vaccine consists of a live Vaccinia virus , which is a "pox"-type virus related to smallpox. There are significant side effects and risks associated with this vaccine, such as skin complications (eczema vaccinatum) which may occur in people with pre-existing eczema, allergic reactions at site of vaccination, vaccinia gangrenosa (or progressive vaccinia), eye infections (spread of virus from site of vaccination), postvaccinal encephalitis, intrauterine vaccinia, and viremia(15). Thus vaccination should be administered only to those exposed to the virus or facing a high probability of exposure(4).

A successful primary vaccination confers full immunity to smallpox in more than 95% of persons for perhaps 5 to 10 years, and successful revaccination probably provides protection for 10 to 20 years or more(4,23). The recently recognised risk of myopericarditis with both first and second generation vaccinia vaccines serves as a reminder that larger-scale studies of newer vaccines are necessary to further define their safety profiles and relative roles in protection against smallpox(24). Two attenuated vaccine strains have also been isolated and tested: modified vaccinia Ankara (MVA), which has been effectively used in more than 1900 people with few adverse effects(25); and a Japanese strain (LC16m8), which is licensed in Japan, and was safely used on more than 50,000 children in the 1970(26). PROPHYLAXIS: Vaccination very early in the incubation period (within 3 to 4 days of exposure) can markedly attenuate or even prevent clinical manifestations of smallpox. Full protection occurs after successful vaccination(4).

Vaccination at 4 to 7 days after exposure likely offers some protection from disease or may modify the severity of disease(23). Unless directly exposed, the vaccine is not advised for groups of people at greater risk for serious side effects, including pregnant and nursing women, children younger than 12 months of age, immuno-compromised patients, HIV patients, or patients with a history of eczema(4,23). Vaccinia immune globulin has also been administered (0.6 ml/kg IM) within 3 days of exposure(4,10). SECTION VI - LABORATORY HAZARDS LABORATORY-ACQUIRED INFECTIONS: Except for a laboratory-associated smallpox death at the University of Birmingham, England, in 1978, no further cases have been identified. All known variola virus stocks are held under security at WHO collaborating centers located at Centers for Disease Control and Prevention, Atlanta, United States, or the State Research Centre of Virology and Biotechnology, Koltsovo, Novosibirsk Region, Russia(1,4). The possession and use of variola viruses are restricted to the WHO collaborating centers(27).

SOURCES/SPECIMENS: Scrapings of skin lesions, scab materials, papular, vesicular, or pustular fluid, crusts, bodily fluids including blood and urine, respiratory secretions, pharyngeal and tonsillar swabbings(1,4,8,19,27). PRIMARY HAZARDS: Ingestion, parenteral inoculation, and droplet or aerosol exposure of mucous membranes or broken skin with infectious fluids or tissues(27). SPECIAL HAZARDS: Genetically engineered recombinant vaccinia viruses pose a potential risk to laboratory personnel, through direct contact or contact with clinical materials from infected volunteers or animals(8,27). SECTION VII – EXPOSURE CONTROLS / PERSONAL PROTECTION RISK GROUP CLASSIFICATION: Risk Group 4(28). CONTAINMENT REQUIREMENTS: Containment Level 4 facilities, equipment, and operational practices for work involving infectious or potentially infectious materials, animals, or cultures. PROTECTIVE CLOTHING: Personnel entering the laboratory must remove street clothing, including undergarments, and jewellery, and change into dedicated laboratory clothing and shoes, or don full coverage protective clothing (i.e., completely covering all street clothing).

Additional protection may be worn over laboratory clothing when infectious materials are directly handled, such as solid-front gowns with tight fitting wrists, gloves, and respiratory protection. Eye protection must be used where there is a known or potential risk of exposure to splashes(29). OTHER PRECAUTIONS: All activities with infectious material should be conducted in a biological safety cabinet (BSC) in combination with a positive pressure suit, or within a class III BSC line. Centrifugation of infected materials must be carried out in closed containers placed in sealed safety cups, or in rotors that are loaded or unloaded in a biological safety cabinet. The integrity of positive pressure suits must be routinely checked for leaks. The use of needles, syringes, and other sharp objects should be strictly limited. Open wounds, cuts, scratches, and grazes should be covered with waterproof dressings. Additional precautions should be considered with work involving animal activities(29). SECTION VIII - HANDLING AND STORAGE SPILLS: Allow aerosols to settle and, wearing protective clothing, gently cover spill with paper towels and apply appropriate disinfectant, starting at the perimeter and working towards the centre. Allow sufficient contact time before clean up (30 min)(29).

DISPOSAL: Decontaminate all materials for disposal by steam sterilisation, chemical disinfection, and/or incineration(29). STORAGE: In leak-proof containers that are appropriately labelled and locked in a Containment Level 4 laboratory(29). SECTION IX – REGULATORY AND OTHER INFORMATION REGULATORY INFORMATION: The import, transport, and use of pathogens in Canada is regulated under many regulatory bodies, including the Public Health Agency of Canada, Health Canada, Canadian Food Inspection Agency, Environment Canada, and Transport Canada. Users are responsible for ensuring they are compliant with all relevant acts, regulations, guidelines, and standards. UPDATED: September 2010. PREPARED BY: Pathogen Regulation Directorate, Public Health Agency of Canada. Although the information, opinions and recommendations contained in this Pathogen Safety Data Sheet are compiled from sources believed to be reliable, we accept no responsibility for the accuracy, sufficiency, or reliability or for any loss or injury resulting from the use of the information. Newly discovered hazards are frequent and this information may not be completely up to date.

Copyright © Public Health Agency of Canada, 2010 Canada This MSDS / PSDS document, provided by Public Health Agency of Canada (PHAC), is offered here as a FREE public service to visitors of MSDSonline. As outlined in this site’s Terms of Use, MSDSonline is not responsible for the accuracy, content or any aspect of the information contained therein. Smallpox - NORD (National Organization for Rare Disorders) Smallpox Variola Minor (Alastrim) General Discussion Smallpox is an infectious disease caused by the variola virus. It is characterized by fever, a general feeling of ill health (malaise), headaches and back pain. These initial symptoms are followed by a rash and small, raised bumps or lesions (pocks) within two or three days. Smallpox was a highly contagious disease, but was declared eradicated in 1980. However, recently smallpox has become of interest because of the possibility of its use as a weapon of warfare or of terrorism. There were two strains of smallpox, variola major and variola minor. Signs & Symptoms Symptoms of smallpox appear after an incubation period of approximately 12 days (7-17 days).

Initial symptoms are a high fever, a general feeling of ill health (malaise), headaches, and back pain often resulting in affected individuals becoming bedridden. In some cases, severe abdominal pain and delirium may also be present. Approximately two to three days after the onset of the disease, a characteristic rash forms on the mucous membranes (mucosa) of the mouth and throat (pharynx) as well as on the face and arms. The rash often spreads to affect the legs and trunk. Within one or two days, small, red bumps or lesions form (papules). These lesions become pus-filled (pustules) sometimes breaking open and resulting in bloody, oozing sores. Eventually, the pustules begin to crust and then scab. In three to four weeks, the scabs fall off, usually leaving scars or pox marks. There were two strains of smallpox. Variola major was more severe and had a fatality rate of approximately 30 percent; variola minor was less severe and had a fatality rate of less than 1 percent.

Life-threatening complications that may be associated with smallpox include blood loss, the presence of toxins in the blood (toxcemia), cardiovascular problems, and/or secondary bacterial infections. Causes Smallpox is caused by infection with the variola virus. The disease is spread through person to person contact, most often from inhalation of droplet nuclei expelled through the back of the throat (oropharynx) of an infected person. Droplet nuclei are small particles containing virus-infected respiratory secretions that are pushed out into the air by coughing or sneezing. These airborne particles may then be inhaled by another person. Individuals with smallpox are most infectious during the first week of illness. Smallpox may also be spread through direct contact with an infected person or through contact with contaminated material such as clothing, blankets or bed linen. Affected Populations Smallpox existed for thousands of years until the World Health Organization declared it extinct in May of 1980. The last known case of smallpox occurred in Somalia in 1977. The only known stores of the smallpox virus are in two locations: the Centers for Disease Control and Prevention (CDC) in Atlanta and Russian government laboratories near Moscow.

However, government officials fear that secret stores may be maintained elsewhere and may be released deliberately as an act of warfare (bioterrorism). Individuals with weakened immune systems such as those with leukemia, lymphoma or AIDS would be more susceptible to smallpox infection if the disease was reintroduced into society. Related Disorders Symptoms of the following disorders can be similar to those of smallpox. Comparisons may be useful for a differential diagnosis: Monkeypox is a rare infectious disease caused by a virus that is related to the virus that causes smallpox. Individuals with monkeypox develop a rash that is similar to one associated with smallpox. Most cases of monkeypox occur in remote villages in Africa where there is close contact with infected animals. The vaccine developed to treat smallpox also protects against monkeypox. Varicella-zoster is an infectious disease caused by a common virus known as herpes virus. During childhood, infection with the varicella virus causes chickenpox . The varicella virus remains viable in body nerve cells for the rest of that individual’s life.

From time to time, the latent virus leaves its nerve cell home and travels down nerve rootlets to the skin, where it produces the typical rash and pain of the syndrome known as shingles. First infection with the varicella virus always causes chickenpox, even in adults who never had the infection as children. Chickenpox is a highly contagious disease characterized by an itchy skin rash and fever. Chickenpox usually begins with mild constitutional symptoms such as a mild headache, moderate fever and discomfort followed by an eruption appearing in itchy groups of flat or elevated spots and blisters, which form crusts. Shingles is a painful localized recurrence of the skin rash during adulthood. The rash consists of small, red spots that become inflamed and develop into small fluid-filled bumps or lesions. These lesions then become pus-filled before crusting and healing. Fever and muscle aches may precede the development of the rash. Before smallpox’s eradication in 1980, mild cases may have been confused with varicella-zoster infection.

(For more information on this disorder, choose varicella-zoster as your search term in the Rare Disease Database.) Standard Therapies In the United States before 1972, children received routine smallpox vaccinations. However, the duration of immunity granted by the vaccination may be only 10 to 15 years, so those who received the vaccine in childhood may no longer be immune to the disease. According to the Centers for Disease Control and Prevention (CDC), the smallpox vaccine can lessen the severity or prevent illness in individuals who are exposed to the smallpox virus if administered within four days after exposure. The smallpox vaccine contains another virus, vaccina, made from the cowpox virus. The United States currently has an emergency supply of the vaccine in storage at the CDC. During 2000 and 2001, the CDC contracted with pharmaceutical companies to manufacture more of the smallpox vaccine for use if smallpox is employed as a terrorism weapon. In rare cases, the administration of smallpox vaccine results in a severe reaction. Such reaction may be treated by a specialty antibody prepared from human plasma that has been approved by the FDA (February 2005).

This vaccine, vaccinia immune globulin intravenous (human) is also known by the trade name VIGIV and is manufactured and marketed by: DynPort Vaccine Company LLC. 64 Thomas Johnson Drive Frederick, Marylan. 21702. There is no specific treatment for individuals infected by smallpox. Treatment is supportive and directed at specific symptoms present in each individual. Supportive therapy may include antibiotics for secondary bacterial infections and/or medications for pain or fever. In 2002, the U.S. Food and Drug Administration (FDA) granted orphan status to the drug poly-ICLC (Hiltonol), to be used as an adjuvant to smallpox vaccination. Hiltonol is manufactured by Oncovir, Inc., a Washington, DC, pharmaceutical corporation. Additional information on this experimental product may be found at Vaxgen, Inc., in partnership with the Chemo-Sero Research Institute in Japan, is developing a smallpox vaccine, LC16m8, that has been licensed for use in Japan for several years. Their goal is to develop a vaccine that is safer than Dryvax, but equally effective.

Investigational Therapies Research is underway to develop new antiviral drugs for the treatment of infectious diseases such as smallpox. Information on current clinical trials is posted on the Internet at All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site. For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office: Tollfree: (800) 411-1222 References TEXTBOOKS Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996: 1765-68. Fields BN, et al. Fields Virology, 2nd ed. New York, NY: Raven Press; 1990:2113-33. Mandell GL, et al. eds. Mandell, Douglas and Bennett’s Principles and Practice of Infectious Diseases. 4th ed. New York, NY: Churchill Livingstone Inc; 1995:1328-29.

JOURNAL ARTICLES Henderson DA, et al. Smallpox as a biological weapon. Medical and public health management. JAMA. 1999;281:2127-37. Georges AJ, et al. Biohazards due to Orthopoxvirus: should we re-vaccinate against smallpox? Med Top (Mars). 1999;59:483-87. Years Published 2001, 2004, 2009 The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional. The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.

National Organization for Rare Disorders (NORD) 55 Kenosia Ave., Danbury CT 06810 • (203)744-0100 Report Index Smallpox - Symptoms, Diagnosis, Treatment of Smallpox - NY Times Health Information Back to Top Causes Smallpox was once found throughout the world, causing illness and death wherever it occurred. It mainly affected children and young adults. Family members often infected each other. Smallpox spreads easily from one person to another from saliva droplets. It may also be spread from bed sheets and clothing. It is most contagious during the first week of the infection. It may continue to be contagious until the scabs from the rash fall off. Researchers believe that the smallpox infection might be able to stay alive (under the right conditions) for as long as 24 hours. In unfavorable conditions, the virus may only remain alive for 6 hours. People were once vaccinated against this disease. However, the United States stopped giving the smallpox vaccine in 1972. In 1980, the World Health Organization (WHO) recommended that all countries stop vaccinating for smallpox.

There are two forms of smallpox: Variola major is a serious illness that can be life threatening in people who have not been vaccinated Variola minor is a milder infection that rarely causes death A massive program by the World Health Organization (WHO) wiped out all known smallpox viruses from the world in the 1970s, except for a few samples saved for government research. Researchers continue to debate whether or not to kill the last remaining samples of the virus, or to preserve it in case there may be some future reason to study it. You are more likely to develop smallpox if you: Are a laboratory worker who handles the virus (rare) Are in a location where the virus was released as a biological weapon It is unknown how long past vaccinations stay effective. People who received the vaccine many years ago may no longer be fully protected against the virus. THE RISK OF TERRORISM There is a concern that the smallpox virus could be intentionally spread through a terrorism attack. The virus could be deliberately spread in spray (aerosal) form. Symptoms usually occur about 12 - 14 days after you have been infected with the virus.

They may include: Backache Raised pink rash -- turns into sores that become crusty on day 8 or 9 Severe headache Back to Top Treatment If athe smallpox vaccine is given within 1-4 days after a person is exposed to the disease, it may prevent illness or make the illness less severe. Once symptoms have started, treatment is limited. There is no drug specifically for treating smallpox. Sometimes antibiotics are given for infections that may occur in people who have smallpox. Taking antibodies against a disease similar to smallpox (vaccinia immune globulin) may help shorten the duration of the disease. People who have been diagnosed with smallpox and everyone they have come into close contact with need to be isolated immediately. They need to receive the vaccine and be monitored. Emergency measures would need to be taken immediately to protect the general population. Health officials would follow the recommended guidelines from the CDC and other federal and local health agencies. Back to Top When to Contact a Medical Professional If you think you may have been exposed to smallpox, contact your health care provider immediately.

Because smallpox has been wiped out this would be very unlikely, unless you have worked with the virus in a laboratory or there has been an act of bioterrorism. Back to Top Prevention Many people were vaccinated against smallpox in the past. The vaccine is no longer given to the general public because the virus has been wiped out. The possible complications and costs of the vaccine outweigh the benefits of taking it. If the vaccine needs to be given to control an outbreak, it can have a small risk of complications. Some complications are mild, such as rashes. Others are more serious. Only military personnel, health care workers, and emergency responders may receive the vaccine today. Smallpox vaccination policies and practices are currently being reviewed. Clinical Trials Review Date: 6/23/2011 Reviewed By: Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School; Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. A.D.A.M., Inc.

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Question: Which disease is also known as Variola?
Answer: SMALL POX