Pellagra: Symptoms, Causes, Tests, and Treatment
HealthcareOnTime
2024-12-08
2024-12-09
3 Min Read
Pellagra, a disease caused by Vitamin B3 deficiency, is a potentially life-threatening but largely preventable condition caused by the decreased levels of niacin in the body. Symptoms seen in cases of niacin deficiency can extend to involve the skin, gastrointestinal system, and cognitive system of an individual. In this article, we’ll attempt to understand what causes pellagra, its risk factors, symptoms, possible complications, and how to treat pellagra.
What is Pellagra?
Pellagra refers to the wide constellation of signs and symptoms seen in an individual with a deficiency of Vitamin B3 (Niacin), either because of the reduced intake of this essential micronutrient or conditions that lead to a functional deficiency of the same.
Niacin, after being absorbed into the body through the intestines, gets converted into nicotinamide adenine dinucleotide (NAD) and nicotinamide adenine dinucleotide phosphate (NADP), coenzymes that are integral to the proper functioning of a wide group of metabolic pathways such as:
- Cellular Functions: include cellular communications pathways and repair of damaged DNA molecules
- Ageing: Diseases associated with ageing are attributed to the decreased levels of NAD in the body1
- Generation of energy molecules known as adenosine triphosphate (ATP)
- Antioxidant Actions: protecting the body from a dangerous group of biomolecules called reactive oxygen species (ROS)
Symptoms of Pellagra / Vitamin B3 deficiency disease
Pellagra, a disease of Vitamin B3 deficiency, is characterised by 4D's—diarrhoea, dermatitis, dementia, and, if left untreated, potentially death. These classical symptoms of pellagra are further elucidated below:
- Dermatological Manifestations: What causes the classical skin signs of pellagra? This can be explained on the basis that in the deficiency of niacin, skin cells become more photosensitive and, hence, more susceptible to damage by the rays of the Sun. Characteristic features include:
- Casal’s necklace: one of the pathognomonic signs of pellagra with a band of hyperpigmented or darkened lesions around the neck. 2
- Dermatitis: Known as one of the characteristic signs of pellagra, seen over sun-exposed areas of the body like forearms and feet, which initially resemble sunburns.
- Anogenital erosions: In some cases of Vitamin B3 deficiency diseases, the skin around the genital area appears to be reddened and macerated.
- Gastrointestinal Manifestations: Caused by the damage to intestinal mucosa leading to a state of irritation and chronic inflammation. These symptoms of pellagra, a disease of Vitamin B3 deficiency, include:
- Nausea, vomiting which may also lead to fatigue and generalised weakness
- Diarrhoea develops in almost half of all reported cases of Vitamin B3 deficiency diseases and is usually the first symptom to develop in pellagra. The diarrheal episodes are unrelenting in nature and may sometimes last for more than 4 weeks.
- Abdominal pain, a non-specific symptom of pellagra
Diseases of Vitamin B3 deficiency may also afflict the mucosal surfaces of the body, leading to the development of certain classical mucosal signs of pellagra. They include glossitis, the inflammation of the tongue, with the tongue appearing to be red and swollen with shallow erosions and ulcers, and angular stomatitis, with inflammation of the oral mucosa, which appears to be red and tender.
One of the possible explanations behind why the effects of niacin deficiency are most noticeable in the skin, and gastrointestinal tract is that they possess the highest fraction of actively dividing cells.
Neuropsychiatric manifestations: In the absence of niacin, nerve cells or neurons do not possess the energy molecules that they badly need for their optimum functioning, which leads to the development of a gamut of neuropsychiatric symptoms of pellagra like:
- Apathy, characterised by a lack of motivation or interest
- Depression, a symptom of niacin deficiency that might require medical attention owing to the faster rates of deterioration in the temperament of a patient
- Delusions
- Difficulty in concentrating on a single task
- Irritability, restlessness
- Dementia, one of the classical symptoms of pellagra described under the 4D’s
Causes of Pellagra
What causes pellagra? The causes of pellagra can be broadly divided into primary and secondary causes. As we’ve discussed before, pellagra is a disease caused by Vitamin B3 deficiency, and hence, the primary cause is due to the decreased intake of niacin in the body. On the other hand, the secondary causes are attributed to disorders that lead to the decreased absorption of niacin into the body. These causes of pellagra include:
- Alcohol abuse—through multiple mechanisms, like causing widespread organ damage, including liver cirrhosis. Bowel disease leads to the development of symptoms of niacin deficiency.
- Hartnup disease is a rare genetic disorder that makes it harder for the body to absorb essential amino acids like tryptophan, which is essential in the formation of niacin.
- Carcinoid syndrome is a rare neuroendocrine tumour that causes overproduction of serotonin, which depresses the secretion of niacin, hence leading to the development of symptoms of pellagra. 3
- Maise-rich diet: Niacin in maise is present in a niacytin form, which cannot be metabolised by our bodies, causing symptoms of niacin deficiency. 4
- Jowar/Sorghum-based diet: increased leucine content that interferes with the metabolism of tryptophan and niacin, hence causing pellagra.
- Iron deficiency anemia: Since iron is one of the micronutrients required for the proper utilisation of niacin, it causes diseases of Vitamin B3 deficiency.
- Drugs: Various categories of drugs known to cause pellagra, like antispasmodics and chemotherapy drugs, negatively influence the metabolism of niacin in the body, whereas some anti-tubercular drugs like isoniazid may disrupt the synthesis pathway. 5
- Gastric bypass surgeries can potentially damage the intestinal mucosa, which plays an integral role in the absorption of essential vitamins like niacin, hence leading to the development of pellagra, an important disease of Vitamin B3 deficiency.
How is pellagra diagnosed?
Most cases of Vitamin B3 deficiency diseases are picked up clinically on the basis of the presence of pathognomonic signs and symptoms. In some cases, they may be simply diagnosed on the basis of improvement of symptoms of pellagra on the administration of niacin supplementation. But your doctor may also rely on other diagnostic modalities to identify what causes pellagra. They include:
- Urine tests: testing for certain chemicals like N-methylnicotinamide to identify if your body has enough stores of niacin
- Blood tests: measurement of the serum levels of niacin, tryptophan, and active forms of niacin like NAD and NADP
When primary causes of Vitamin B3 deficiency disease have been ruled out, the next question remains: what else can cause pellagra? The secondary causes of pellagra, like carcinoid tumour, liver cirrhosis, and other gastrointestinal disorders, will have to be kept in mind.
Treatment options for Vitamin B3 deficiency/Pellagra
Since pellagra is primarily a disease caused by Vitamin B3 deficiency, the answer for how to treat pellagra lies in the supplementation of niacin or nicotinamide in either oral or parenteral formulations. Most, if not all, patients of primary pellagra start to show signs of improvement in symptoms of pellagra within a few days of starting with supplements and other dietary modifications. Skin lesions may take a little longer to completely resolve, whereas neurological symptoms of pellagra, like dementia, might prove to be irreversible.
In cases of secondary pellagra, the road to recovery might be slightly longer and is not as straightforward as in cases of primary pellagra since the focus of treatment in such cases of Vitamin B3 deficiency here lies in treating the underlying cause. However, some patients tend to get better with niacin/nicotinamide supplementation alone.
There aren’t any major side effects that are reported due to the usage of niacin or nicotinamide supplements to counteract the effects of niacin deficiency. However, some patients with Vitamin B3 deficiency diseases who are on supplements for the same may experience skin flushing, episodes of indigestion, headaches, and, in some rare cases, dizziness. If you experience any of these symptoms while on supplements as a form of treatment for Vitamin B3 deficiency, contact your physician immediately.
Prevention of Pellagra
As the old adage goes, prevention is better than cure, which holds true in the treatment of cases of Vitamin B3 deficiency as well. A well-balanced diet that incorporates niacin in its recommended daily allowance can go a long way in preventing the effects of niacin deficiency. This recommended daily allowance of niacin tends to vary slightly with age, gender and other special circumstances, as listed below: 6
- 6 milligrams per day in cases of children between the ages of 1-3 years
- 8 milligrams per day in cases of children between the ages of 1-8 years
- 12 milligrams per day in cases of boys between the ages of 9-13 years
- 16 milligrams per day in cases of boys between the ages of 14-18 years
- 12 milligrams per day in cases of girls between the ages of 9-13 years
- 14 milligrams per day in cases of girls between the ages of 14-18 years
- 16 milligrams per day in cases of men between the ages of 19-70 years
- 14 milligrams per day in cases of women between the ages of 19-70 years
- 18 milligrams per day in cases of pregnant women between the ages of 19-50 years
- 17 milligrams per day in cases of lactating women between the ages of 19-50 years
Some of the rich sources of niacin that can be incorporated into the daily diet of individuals to prevent diseases of Vitamin B3 deficiency are 7
- Animal products like beef and chicken liver, chicken breast, turkey and pork
- Seafood like tuna, salmon, and anchovies
- Brown rice
- Whole wheat
- Vegetables like potatoes, green peas
Complications of Untreated Pellagra
Pellagra, a disease of Vitamin B3 deficiency, if left untreated, can lead to a wide range of complications, some of which could be potentially fatal. These include:
- Dementia: A classical symptom of pellagra indicating deterioration of the body. With chronicity, pellagra is known to cause irreversible neurological damage, which may manifest as deficits in memory, problems with coordination of movement, and muscle tremors, among others.
- Skin Sores: In some acute cases, the skin lesions may undergo ulceration and get infected, leading to the development of secondary infections. These infections could lead to fatal septicemia, causing widespread organ damage in rare instances.
- Malnutrition and Cachexia: Characterised by generalised wasting of the body, one of the deteriorating signs of pellagra.
- Death: Due to interminable nerve damage in vital organs of the body and, rarely, septic complications caused by the infection of skin lesions.
Conclusion
Though the incidence of Pellagra or Vitamin B3 deficiency disease in Western countries like the United States is reported to be less than 1%, thanks to innovations in food fortifications, it’s still a troubling health concern in developing countries like India and China owing to the rampant rates of undernutrition. While pellagra is a potentially life-threatening condition, it does respond well to nutritional supplementation in cases of primary pellagra, while the secondary causes of pellagra are a little harder to treat successfully. Look out for warning signs of pellagra, whether it be cognitive deficits, characteristic skin rashes, or unrelenting episodes of diarrhoea. It may be prudent to seek the help of a health professional if you’re encountering such symptoms attributable to niacin deficiency.
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