The 3 Signs of Pellagra and How to Treat Them - Verywell Health

Pellagra is a condition that occurs when a person develops a deficiency in vitamin B3, also called niacin. There are two types of deficiency a person can have. A primary deficiency occurs when a person is not getting enough niacin in their diet, and a secondary deficiency is when the body isn't able to use the nutrient properly.

Pellagra shows up a collection of symptoms, commonly referred to as "the three D's": dementia, diarrhea, and dermatitis. While the condition can be cured if diagnosed, pellagra can be fatal if left untreated.

Verywell / Nusha Ashjaee 

The Three D's of Pellagra

There are three common symptoms of pellagra referred to as the "three D's." Untreated pellagra will lead to death, sometimes called the "fourth D," within a few years.

Diarrhea

In the early stages, pellagra symptoms may be vague. Patients with the condition may only have one or two of the "Ds" along with other nonspecific symptoms. The rash, however, is seen in the majority of cases.

Watery diarrhea is usually the first symptom and is the result of inflammation of the gastrointestinal mucosa. As the condition progresses, people with pellagra may have no appetite (sometimes leading to anorexia), feel sick to their stomach (nauseated), or have stomach pain. These symptoms can cause a cycle where a person who is already malnourished does not feel well enough to eat.

Dermatitis

Dermatitis, or skin inflammation, typically appears in the form of a rash in sun-exposed areas of the body. In fact, it often looks like a sunburn. The rash may become more intense, with skin peeling and changes in skin color (pigmentation) that can be permanent.

As the rash begins to blister, the skin may become rough and scaly, especially on the bottom part of the hands and feet, as well as on the face (a pattern known as a butterfly or malar rash). Areas of affected skin may be itchy, painful, and sensitive to the sun.

In the early stages, pellagra symptoms may be vague. Patients with the condition may only have one or two of the "Ds" along with other nonspecific symptoms. The rash, however, is seen in the majority of cases.

Pellagra on neck. DermNet / CC BY-NC-ND 

Dementia

The neurological signs and symptoms occur later when the deficiency has become severe and has been going on for a prolonged period of time. At first, a person may experience memory loss, trouble sleeping, and mood changes.

As the deficiency gets worse, the neurological symptoms can be severe: a person may become disoriented or not know where they are, begin to have hallucinations, or even be in a stupor, failing to respond to their surroundings.

In extreme cases, the neurological symptoms of pellagra appear dementia-like or even stroke-like.

As the condition worsens and body systems begin to shut down, other symptoms may develop, including:

• Loss of coordination
• Glossitis (tongue swelling that can obstruct a person's airway)
• Dementia
• Stupor
• Coma

Left untreated, pellagra can be fatal in three to five years.

What Causes Pellagra?

Primary and secondary pellagra have different underlying causes. Primary pellagra occurs when a person is low in niacin or tryptophan.

However, in some cases, even though a person is getting enough niacin from the food they eat, their body is not able to use it properly. This may be due to an underlying condition or caused by a medication. Reasons people develop secondary pellagra include:

• Genetic conditions like Hartnup disease, which inhibits the body's ability to absorb tryptophan from the intestines
• Treatment with drugs that affect how the body uses B vitamins, such as the antituberculosis drug isoniazid or phenobarbital
• Types of tumors that produce excessive serotonin (carcinomas) leading to a condition called carcinoid syndrome
• Adhering to very restricted diets ("fad diets") or maize-based diets that have not been nixtamalized

Additionally, conditions that put stress on the body (thereby increasing nutritional needs) can also lead to pellagra. Patients may be at increased risk of developing the deficiency if they have:

• Human Immunodeficiency Virus (HIV)
• Anorexia nervosa
• Chronic alcoholism and/or drug abuse
• Liver cirrhosis
• Patients receiving long-term dialysis

A person's socioeconomic circumstances can also place them at risk for developing pellagra. People living in poverty who don't have adequate access to nutritious food, those living in famine conditions, and refugees are more likely to become severely malnourished.

While pellagra mostly develops in adults, children who are living in countries experiencing famine, whose families are homeless, or who are being neglected may also be at risk.

Pellagra occurs equally in men and women. It primarily occurs in adults but in some cases, infants and children may be at risk. People of all races can develop pellagra, though it is seen more often in developing countries and poverty-stricken regions of developed nations.

Diagnosing Pellagra

If a healthcare provider suspects a person has pellagra, there are several ways to confirm the diagnosis. They will perform a physical exam and ask the person about diet and lifestyle.

Findings that support a clinical diagnosis of pellagra include the characteristic skin rash. Patients often report diarrhea or other gastrointestinal symptoms, including swollen tongue. In some cases, patients will have started to experience changes in their moods, sleeping habits, and cognition.

Pellagra is uncommon in children, but it does occur. Younger patients may not present with the typical symptoms of pellagra, however, which can make diagnosis tricky.

Usually, children who are found to have pellagra are also suffering from other nutritional deficiencies, either as a result of socioeconomic circumstances or an underlying illness.

To confirm the diagnosis of pellagra, doctors may want to take blood or urine samples to test for low levels of niacin. They may also be looking for other abnormalities, such as low tryptophan, NAD, and NADP levels. Tests can also help rule out other conditions that have similar symptoms.

If a person is found to have pellagra, the next step will be determining the cause. Sometimes, the answer will be fairly obvious, such as if someone reports eating a limited diet or is malnourished.

Other patients may need additional tests to determine the reason for the deficiency. These tests may include:

• Additional blood tests to check for infections or markers of certain illnesses
• Urine tests to see how much niacin a person is excreting
• Imaging, such as an X-ray or CT scan, to assess the organs or look for tumors
• Special tests, like a colonoscopy, to look at the gastrointestinal system if a condition like Crohn's is suspected
• Examining samples from skin lesions under a microscope

Diagnosing pellagra and figuring out what caused a person to develop the condition is very important. It can also cause a number of complications, including:

• Mental changes, such as confusion, depression, and anxiety
• Trouble swallowing due to swelling of the tongue and inflammation
• Hallucinations and delusions
• Permanent changes to the skin's texture and color

Untreated niacin deficiency can cause a person to go into a coma. After several years, the condition causes major damage to the internal organs, which is ultimately fatal.

How Is Pellagra Treated?

The main treatment for pellagra is nicotinamide, a water-soluble form of niacin, for 3–4 weeks. This form causes fewer side effects than regular niacin.

Treatment may also include other nutrients to aid in recovery. A high-calorie, nutritious diet with plenty of hydration is essential to helping the body heal and to prevent a recurrence of pellagra.

The first line of treatment for someone with pellagra is ensuring they have enough to eat, and that the diet they are eating is well-balanced and adequate in calories.

Treatment may also include addressing underlying problems such as chronic alcohol dependence.

Once a person is able to resume a diet of solid foods, they may work with a dietician or nutritionist to ensure they are getting plenty of niacin in their diet. Meat, dairy, and nuts are good sources of niacin, as are green, leafy vegetables.

People can also take a supplemental form of vitamin B3, especially if they are also deficient in other B vitamins (such as in the case of malabsorption) or are taking medications that affect how their body can use niacin. A healthcare provider can help a person decide what regimen they need.