Pellagra

Introduction

What is pellagra?

Pellagra is the late stage of severe niacin deficiency. Niacin, or vitamin B-3, a water-soluble vitamin. Pellagra is a disease caused by deficiency of niacin (one of the B complex vitamins) or tryptophan (an amino acid). An amino acid is a building block for proteins. Pellagra is defined by the systemic disease resulting from niacin deficiency, and it is characterized by diarrhoea, dermatitis, dementia, and death, which usually appear in this order. GI tract symptoms always precede dermatitis, or, "Pellagra begins in the stomach". The full tetrad of symptoms is usually not well developed in infants and children.

Pellagra is observed in malnourished individuals and as a complication of isoniazid therapy; however, the diagnosis is often overlooked or delayed, occasionally with life-threatening consequences.

Causes of Pellagra

Causes of pellagra

• Inadequate diet - lack of animal protein. Found prevalent in areas where people consume large amounts of maize meal and corn
• Certain intestinal diseases
• Alcoholism

Secondary pellagra occurs when adequate quantities of niacin are present in the diet, but other diseases or conditions interfere with its absorption and/or processing. Such conditions include the following: 

• Prolonged diarrhea 
• Long-term alcoholism 
• Chronic colitis, particularly colitis ulcerosa 
• Ileitis terminalis 
• Cirrhosis of the liver 
• Tuberculosis of the GI tract 
• Malignant carcinoid tumor
• Hartnup syndrome

Symptoms of Pellagra

• Diarrhoea with associated inflammed red tongue
• Dermatitis: red, scaly lesions in sun exposed regions which become darker with time 
• Dementia: impressions as being painful, annoying bright lights, odours intolerance causing nausea and vomiting, dizziness after sudden movements, restlessness, tense and a desire to quarrel, depression and suicidal thoughts
• Death : within 4-5 years if left untreated
• Butterfly sign 
• A rash on the front of the neck (Casal's necklace). 
• A rash (dermatitis) which is on both sides of the body, and on skin normally exposed to sunlight is a sign of pellagra. Check the face, neck, hands, arms and legs. 

Clinical evidence of pellagra develops 50-60 days after starting a niacin-deficient diet. Early symptoms include diminished appetite, weight loss and weakness.

Prevention and treatment

Vitamin supplementation

Aggressive Vitamin B complex supplementation.

Dietary Intervention 

• To prevent and/or treat pellagra, prescribe a protein-rich diet with adequate calories. The addition of meats, milk, peanuts, leafy green vegetables, whole or enriched grains, and brewer's dry yeast can enhance niacin intake. 
• In patients with oral dysphagia secondary to glossitis, a liquid or a semisolid diet may be required. The long-term inclusion of milk, meat, and eggs in the diet ensures the dietary adequacy of the proteins essential for recovery. 

Activity

Bed rest is mandatory in treating patients with severe pellagra. Patients should avoid sun exposure during the active phase of the disease.

Prevention

Primary prevention is as follows:

• The primary prevention of pellagra involves an adequate diet. 
• Food sources of niacin and/or tryptophan include nutritional yeast, eggs, bran, peanuts, meat, poultry, fish with red meat, cereals (especially fortified cereals), legumes, and seeds. 
• The recommended daily allowance of niacin for infants is 5-6 mg; for children, 9-13 mg; for adults, 13-20 mg; for pregnant women, 17 mg; and for breastfeeding women, 20 mg. 
• Optimal supplementation is 20-30 mg daily. 

Secondary prevention is as follows:

• Patients should avoid sun exposure during the active phase of the disease. 
• Patients should follow a convenient dietary regimen. 
• Close dietary follow-up after the patient's recovery helps prevent the recurrence of pellagra. 

Complications

Dermatitis of pellagra can be distressing and disfiguring. Denudation of the vesiculated and blistered skin lesions can potentially become infected secondarily. Severe glossitis causes dysphagia. GI tract involvement leads to a mal-absorptive state. Depression, anxiety, delusions, hallucinations, and coma are the neuropsychiatric complications observed in patients with pellagra. The malnourished state associated with pellagra results in death if untreated.

How much niacin do you need?

Recommended Dietary Allowance (RDA) for Niacin (8)

*NE, niacin equivalent: 1 mg NE = 60 mg of tryptophan = 1 mg niacin 

Niacin rich foods

*A 3-ounce serving of meat is about the size of a deck of cards.

Information Source Links

• Hegyi J, Schwartz RA, Hegyi V. Pellagra: dermatitis, dementia, and diarrhea. INT J DERMATOL. Jan (2004). 43(1):1-5. 
• Rajakumar K. Pellagra in the United States: a historical perspective. SOUTH MED J. Mar (2000) ;93(3):272-7. 
• Rille JH. Medizinische Gesellchaft Leipzig. Sitzug vom 16. November (1926). Ueber Pellegra (mit Lichtbildern). DERMATOL WOCHENSCHRIFT. 1927;6:189.