What is Pellagra?

By SWA

Pellagra is a nutritional deficiency disease caused by a lack of vitamin B3 (niacin) or its precursor, tryptophan, in the diet. It primarily affects populations with diets deficient in niacin or tryptophan, and it is most commonly associated with poverty, malnutrition, and regions where untreated maize (corn) is a staple food. If left untreated, pellagra can become severe and life-threatening.

Pellagra is classically characterized by the "3 D's":

  • Dermatitis
  • Diarrhea
  • Dementia

In extreme, untreated cases, it can lead to a fourth "D"—Death.

Causes of Pellagra

  1. Primary Pellagra: Dietary Deficiency

    • Occurs when there is an inadequate intake of niacin or tryptophan (an amino acid that the body can convert into niacin).
    • Common in areas where maize (corn) is the staple food unless it is treated with an alkali (a process called nixtamalization) to release niacin in a bioavailable form.
    • Diets low in animal products or niacin-rich plant foods increase the risk (e.g., poorly planned vegan diets).

  2. Secondary Pellagra: Impaired Niacin Metabolism or Absorption

    • Chronic Alcoholism: Reduces niacin absorption and increases excretion.
    • Malabsorption Disorders: Conditions like Crohn’s disease, celiac disease, or chronic diarrhea impair the absorption of niacin.
    • Carcinoid Syndrome: Excess serotonin production depletes tryptophan, leaving less available for niacin synthesis.
    • Hartnup Disease: A genetic disorder that reduces the absorption of tryptophan.
    • Medications: Drugs like isoniazid, 5-fluorouracil, and certain anticonvulsants can interfere with niacin metabolism.
    • Increased Requirements: Pregnancy, breastfeeding, or severe infections can increase the body’s need for niacin.

Symptoms of Pellagra

Pellagra presents with systemic symptoms that progress if untreated. These symptoms primarily involve the skin, gastrointestinal (GI) tract, and nervous system.

1. Dermatitis

  • A symmetrical, scaly rash appears on areas exposed to sunlight, such as the face, neck, hands, and forearms.
  • The rash is characterized by redness, roughness, and cracking of the skin. Over time, it can darken, thicken, and become hyperpigmented.
  • This rash is often referred to as Casal's necklace when it appears around the neck.

2. Diarrhea

  • Gastrointestinal inflammation leads to:
    • Watery diarrhea
    • Nausea
    • Abdominal pain
  • Prolonged diarrhea contributes to malnutrition and weight loss.

3. Dementia

  • Neurological and psychiatric symptoms develop, including:
    • Irritability
    • Depression
    • Anxiety
    • Confusion
    • Memory loss
    • Hallucinations
    • Delusions or psychosis in advanced cases

4. Other Symptoms

  • Glossitis: Swollen, red, and painful tongue.
  • Cheilitis: Cracks and fissures at the corners of the mouth.
  • Fatigue and general weakness.

Complications of Pellagra

If left untreated, pellagra can cause:

  • Severe Dehydration: Due to chronic diarrhea.
  • Malnutrition: Weight loss and weakness worsen over time.
  • Chronic Neurological Damage: Prolonged dementia may become irreversible.
  • Death: Untreated pellagra is fatal, often due to complications like severe malnutrition, dehydration, or infections.

Diagnosis of Pellagra

  1. Clinical Presentation:

    • Diagnosis is primarily based on the characteristic triad of dermatitis, diarrhea, and dementia.
    • A dietary history to assess niacin and tryptophan intake is crucial, especially in at-risk populations.

  2. Response to Niacin Treatment:

    • Rapid improvement of symptoms (e.g., rash and diarrhea) after niacin supplementation is diagnostic.

  3. Laboratory Tests (Rarely Used):

    • Urinary levels of niacin metabolites (e.g., N-methylnicotinamide) can confirm deficiency.
    • Blood levels of niacin or tryptophan may be assessed in some cases.

Treatment of Pellagra

Treatment of pellagra involves addressing the underlying niacin deficiency and associated symptoms.

  1. Niacin or Nicotinamide Supplementation:

    • Mild Deficiency: 50–100 mg/day of nicotinamide or niacin orally.
    • Severe Cases: 300–500 mg/day, often divided into multiple doses.
    • Nicotinamide is preferred because it does not cause the skin flushing associated with high doses of niacin.

  2. Dietary Interventions:

    • Encourage a balanced diet rich in niacin and tryptophan:
      • Meat (e.g., chicken, fish, liver)
      • Whole grains, legumes, and peanuts
      • Fortified cereals and bread
      • Mushrooms and green leafy vegetables

  3. Treat Underlying Causes:

    • Manage malabsorption conditions, chronic infections, or alcoholism.
    • Adjust medications that interfere with niacin metabolism.

  4. Supportive Care:

    • Hydration for diarrhea.
    • Psychiatric care for severe mental health symptoms.

Prevention of Pellagra

  1. Niacin-Fortified Foods:

    • In many countries, grains, cereals, and flours are fortified with niacin to prevent pellagra.

  2. Adequate Diet:

    • A balanced diet that includes niacin-rich foods is key, especially for populations relying on maize or other poor dietary staples.

  3. Education:

    • Public health campaigns to educate at-risk populations on proper food preparation (e.g., nixtamalization of corn) and balanced nutrition.

  4. Supplementation for At-Risk Groups:

    • Populations at higher risk (e.g., alcoholics, individuals with malabsorption, vegans, or pregnant women) may require niacin or multivitamin supplements.

Conclusion

Pellagra is a preventable and treatable disease caused by vitamin B3 deficiency. While it is rare in developed countries, it remains a significant health issue in populations with limited access to niacin-rich foods or where untreated maize is a dietary staple. Early diagnosis and treatment with niacin supplementation and dietary improvements can rapidly resolve symptoms and prevent complications. Prevention strategies, such as food fortification and education, are crucial for reducing the global burden of pellagra.


© 2000-2025 Sieglinde W. Alexander. All writings by Sieglinde W. Alexander have a fife year copy right.
Library of Congress Card Number: LCN 00-192742
ISBN: 0-9703195-0-9

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