Pellagra is a hypovitaminosis due to a deficiency of vitamin B3 (niacin). It is usually the result of malnutrition or undernutrition. However, there is also a genetic form of pellagra called Hartnup disease.
What is pellagra?
Pellagra represents an undersupply of vitamin B3 (niacin, nicotinic acid) to the body. Today, this condition plays a major role only in poor countries with frequent famines and in countries where corn is the main food. In corn, niacin is present only in bound form. Only in alkaline digested corn does niacin become usable by the body. After corn was introduced as a foodstuff in Europe following the discovery of America by Christopher Columbus, a strange disease spread, the leading symptom of which was rough skin. In addition, there were other physical and psychological symptoms. It was already suspected that the corn must have had something to do with this disease. However, it was speculated whether possible specific plant toxins or mold infestation could be the cause of this disease. Little knowledge of proper nutrition contributed to the occurrence of veritable pellagra epidemics in the 18th and 19th centuries.
Causes
The cause of pellagra is mainly an undersupply of nicotinic acid to the body. Nicotinic acid, also known as vitamin B3 or niacin, is abundant in meat, liver, fish, and whole grain products. Milk and dairy products also contain a lot of vitamin B3. In corn or sorghum millet, niacin is initially present in a form that cannot be utilized. It is firmly bound in the molecule and can only be released by alkaline treatment of these foods. A deficiency of vitamin B3 therefore occurs in unbalanced diets with untreated corn or sorghum millet. However, niacin can also be synthesized in the body from the amino acid tryptophan. If the unbalanced diet is additionally associated with a protein deficiency, a particularly pronounced deficiency of niacin therefore also results. Niacin is involved in numerous metabolic processes in the organism. It plays an important role in energy production from carbohydrates, fats and proteins. Furthermore, it participates in the formation of skin, muscle or nerve cells and in the repair of genetic material. Thus, due to a deficiency of niacin, errors occurring in DNA and RNA during cell renewal processes can no longer be sufficiently corrected. Furthermore, niacin also improves memory through its influence on the nervous system. Therefore, the deficiency of niacin causes a complex of symptoms known as pellagra.
Symptoms, complaints, and signs
Pellagra is manifested by the appearance of many different symptoms. This is due to the central role that niacin plays in metabolism. The leading symptom is roughening of the skin. There is itching, reddening of the skin, thickening of the skin, browning of the skin,
inflammation of the mucous membranes in the digestive tract and nerve damage. Typical symptoms are diarrhea, dermatitis and dementia. Furthermore, the tongue turns black. In addition, there is pain in the limbs, fatigue, fever, headaches, cramps, tremors, paralysis and mental disorders. In severe cases, the disease can lead to death within weeks. Often, due to a general malnutrition, a niacin deficiency is also associated with a deficiency of other vitamins. Therefore, other symptoms are often added to the typical symptoms of pellagra.
Diagnosis and course of the disease
Because pellagra is extremely rare in Europe today, the diagnosis is not made in the vast majority of cases despite typical symptoms. Many diseases may present with similar symptoms. Only in the context of conspicuous malnutrition, such as in anorexia, can these symptoms lead to the suspicion of niacin deficiency. This suspicion should be confirmed by a determination of niacin and its degradation products in the urine. However, there is also a genetically determined metabolic disease which is characterized by an extreme deficiency of niacin. This is the so-called Hartnup disease. If pellagra-like symptoms occur without a nutritional cause, the amino acid concentration should be determined by urinalysis.Since Hartnup’s disease is characterized by the body’s inability to retain the amino acids of degraded proteins in the blood, there is a high concentration of amino acids in the urine. Genetic analysis can confirm the diagnosis.
Complications
A deficiency of vitamin B3 is first noticeable by skin that has become rough, itching, and a reddening or browning of the skin. Because vitamin deficiency is virtually nonexistent in the Western world, pellagra often goes undiagnosed even when all the typical symptoms are present. If the vitamin deficiency is not corrected, a number of complications can occur. Mucosal inflammation of the digestive tract is common. The general performance capacity decreases. The affected person develops symptoms that are also typical of a cold. In particular, he suffers from fatigue, headache and fever. The skin becomes noticeably worse. Dental health also suffers from a persistent lack of vitamins. The gums in particular become severely inflamed, and the patient develops severe gingivitis, which can lead to tooth loss. In an advanced stage, damage to the nerves and brain sets in. The patient can no longer concentrate, memory deteriorates steadily. In extreme cases, dementia develops. If the underlying disease remains undiagnosed, the patient’s life is in acute danger. This is especially true if pellagra is due to malnutrition, for example due to anorexia, as these patients usually also have other nutrient deficiencies and a weakened immune system.
When should you see a doctor?
Symptoms such as headaches, intestinal disorders or cramps should be promptly clarified by a doctor, as they indicate pellagra or another disease caused by a vitamin and nutrient deficiency. A visit to the doctor is recommended at the latest with skin changes, inflammation of the mucous membranes and neurological complaints. If left untreated, pellagra can lead to diseases such as dementia or dermatitis, or even death. People who eat a one-sided diet, especially corn or millet, are particularly likely to develop pellagra. The genetic defect underlying Hartnup’s disease may also be causative for the disease. Patients at risk, such as those who fast regularly or eat a one-sided diet due to a mental illness, should consult their family physician if they suspect a secondary disease. If other risk factors are present, a doctor should be consulted quickly if symptoms are mentioned. The first port of call is the family doctor, the gastroenterologist and the dermatologist if skin complaints are among the symptoms. Routine examinations can be performed by a general practitioner. Any injections are usually administered as an inpatient.
Treatment and therapy
Treatment of pellagra is straightforward. The deficiency of niacin can be well compensated by a diet of meat, fish, liver, whole grains, or dairy products. In cases of extreme niacin deficiency, nicotinic acid can also be given initially. Brewer’s yeast is also well suited to correct the vitamin deficiency. Nicotinic acid deficiency is also frequently treated with additional administrations of tryptophan. If the niacin deficiency is genetic, as in Hartnup’s disease, substitution therapy with nicotinamide is given. In this case, the health condition usually improves. Sometimes, however, substitution remains ineffective. However, since nicotinamide is toxic to the liver, it is preferable to use niacin in large doses. The subsequent red coloration of the skin disappears after a few weeks of treatment. Parallel to substitution, a high-protein diet with dairy products, poultry, beef, nuts and potatoes is recommended. In contrast to classic pellagra, this high-protein, high-tryptophan diet should be maintained throughout life in Hartnup disease.
Outlook and prognosis
The disease called pellagra, which is based on a chronic niacin or vitamin deficiency, should in itself be able to be eradicated by an adequate dietary supply of fruits and vegetables. However, only economically highly developed countries benefit from the abundance of such foods. Climate change is causing more and more parts of the earth to become karst. Other parts of the planet are increasingly threatened by flooding. Both of these factors are exacerbating the food situation – and this means that pellagra cannot be made to disappear either.The global economic and ecological prognosis must be considered here, as well as the health prognosis. When pellagra occurs, survival depends crucially on whether or not treatment is given immediately. In addition, the duration and severity of the niacin deficiency will determine whether pellagra can still be successfully treated. If the general health is still robust, and the age of the affected person is not too high, pellagra can often be treated successfully. On the other hand, the prognosis is poor if treatment is not prompt. The worse it is in terms of the other parameters mentioned above, the poorer the prospects for complete recovery. Because medical care in many regions of the world affected by pellagra is far from good, many deaths have been caused by pellagra.
Prevention
Pellagra is very rare in Europe because the diet contains sufficient niacin. However, to prevent this disease, an extreme one-sided and low-protein diet should be avoided. Because pellagra today occurs mainly in the context of morbid anorexia, it is important to identify and treat the causes of this eating disorder.
Follow-up
The best way to prevent pellagra is to eat a varied diet. People should avoid a one-sided diet consisting of corn and millet products. Instead, the consumption of eggs, peanuts and meat is useful. They contain nicotinic acid. This self-responsible aftercare usually ensures that the typical symptoms subside. In the case of a mild form of the disease, the physician provides his patient with appropriate knowledge. As a result, no further complications are likely to occur. Pellagra can also be caused by a genetic defect. In this case, permanent follow-up care is necessary because the symptoms may recur. A diet is required. The patient starts a therapy with nicotinic acid or nicotinamide. The doctor and patient arrange follow-up appointments depending on the severity of the symptoms. These serve to discuss the current condition. A physical examination and an analysis of the blood and urine allow conclusions to be drawn about the current condition. Especially in Europe, pellagra raises the suspicion of deliberate malnutrition, such as occurs in anorexia. This is because the disease is unlikely to occur with a normal diet. If such a suspicion is confirmed, psychotherapy may be indicated. The success of the follow-up is then strongly dependent on the extent to which the patient is willing to move away from a certain ideal of beauty.
What you can do yourself
Pellagra is a vitamin B3 deficiency disease caused solely by a deficiency of vitamin B3 (niacin). In advanced stages, immediate intake of niacin in the form of nicotinic acid or nicotinamide is required. If the vitamin deficiency is not caused by malnutrition but by impaired absorption capacity of the small intestine, intravenous supply is recommended instead of oral administration. In the case of a less pronounced disease, everyday and self-help measures consist in the consumption of foods with a high content of niacin. For non-vegetarians, meat and offal of pork and beef as well as salmon and herring are recommended, as are dairy products and egg dishes. The advantage of vitamin B3 derived from animal products is its good absorbability, because the niacin is usually in the easily utilizable form of nicotinamide. But also for vegetarians and even for vegans, nature has foods with a high vitamin B3 content ready. In detail, these are various whole-grain cereals, walnuts, peanuts and dried apricots. A food with a particularly rich content of vitamin B3 is the freshwater algae Spirulina platensis. If the vitamin deficiency is due to impaired absorption capacity in the small intestine, it should be checked whether the deficiency is caused by taking certain medications or by chronic inflammation of the intestinal mucosa or by chronically excessive alcohol consumption.
