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Pellagra

Niacin Deficiency Disease

Pellagra is a systemic disease caused by a deficiency in the B vitamin niacin (also known as vitamin B3 or nicotinic acid). However, it can also be brought about by poorly-balanced intake of amino acids. Pellagra is characterized by the classical symptoms of dermatitis, diarrhea and dementia.


Presentation

Pellagra is characterized classically by the triad of dermatitis, diarrhea and dementia.

  • The dermatitis is essentially a photosensitive rash. It is typified by bilaterally symmetrical erythema at sites of sun exposure, resembling sunburn. Vesicles and bullae can also be seen and can become exudative, crusted and desquamating. Casal's necklace (named after Don Gaspar Casal) describes a symmetrical, desquamating rash in the neck area. The mucous membranes can also be involved with stomatitis of the tongue and mucositis of the urethra and vagina occurring. Stomatitis of the tongue is a sign of acute deficiency.
  • The gastrointestinal symptoms are anorexia, nausea, abdominal pain and diarrhea. The diarrhea is generally watery but can be bloody and mucoid.
  • The neurologic manifestations are varied and can include depression, hallucinations, psychosis, memory loss and may progress to disorientation and delirium.
Malnutrition
  • Pellagra due to malnutrition occurred in an Ottawa women in 1973. She was given a normal diet with supplements of riboflavin, thiamine, niacin and ascorbic acid. The clinical response was striking.[ncbi.nlm.nih.gov]
  • Medical history noted chronic alcoholism, malnutrition, and poor self-care. Evaluation in the hospital suggested that his rash and neurologic changes were a result of malnutrition and vitamin deficiency.[ncbi.nlm.nih.gov]
  • Higher anaemia and hypoalbuminemia frequencies associated with lower serum potassium levels suggest increased protein malnutrition prevalence among pellagrins.[ncbi.nlm.nih.gov]
  • Pellagra is a disease largely associated with alcohol abuse, poverty, and malnutrition and is very common in developing countries.[ncbi.nlm.nih.gov]
  • After treatment with nicotinamide the patient made good progress with a complete resolution of the signs of pellagra and protein malnutrition.[ncbi.nlm.nih.gov]
Anemia
  • Anemia. Dietary management for Pellagra Avoid: . Avoid alcohol consumption. . Avoid excess consumption of maize (corn) and sorghum (jowar), they hinder in absorption of niacin. Consume: .[specialityclinic.com]
  • Other vitamin deficiency diseases besides scurvy and pellagra include rickets, a bone disease caused by too little vitamin D; night blindness, or difficulty seeing in dim light, which is caused by a lack of vitamin A; and pernicious anemia, a condition[pbslearningmedia.org]
  • The diagnosis of Pellagra may involve: Complete evaluation of medical history along with a thorough physical examination Blood tests such as: Complete blood count (which may indicate anemia) Serum protein (levels may be low) Serum calcium (higher levels[dovemed.com]
Weight Loss
  • She reported a 45-kg (100-lb) weight loss during the past four months and no diarrhea or neurologic symptoms.[nejm.org]
  • Early symptoms of vitamin B1 deficiency include tiredness, irritability, poor memory, sleep problems, weight loss and stomach pain.[explorevitamins.co.uk]
  • Early symptoms include diminished appetite, weight loss and weakness. Vitamin supplementation Aggressive Vitamin B complex supplementation. Dietary Intervention To prevent and/or treat pellagra, prescribe a protein-rich diet with adequate calories.[infonet-biovision.org]
  • Closer examination of the patient’s history revealed a 30-lb weight loss from 6 months of emesis, abdominal pain, diarrhea, dysgeusia, and intermittent dysphagia.[consultant360.com]
  • Unusual Clinical Scenarios to Consider in Patient Management The increasing number of weight loss surgeries being performed in the developed countries is leading to a rise in nutritional deficiencies.[clinicaladvisor.com]
Fever
  • The choice of Goldberger itself probably reflected that view, as he had earlier distinguished himself in epidemics of indisputable infectious diseases – yellow fever in Cuba, dengue fever in Texas, and typhus in Mexico City.[blogs.creighton.edu]
  • ‘The troops suffered from malaria, dengue fever, beriberi, hookworm and pellagra.’[en.oxforddictionaries.com]
  • A civil war indeed is like the heat of a fever, but a foreign war is like the heat of exercise, and serveth to keep the body in health, for in a slothful peace both courages will effeminate and manners corrupt. ‎[books.google.ro]
Diarrhea
  • It is a clinical syndrome characterized by four "D's": diarrhea, dermatitis, dementia and ultimately death. We describe a case of pellagra as the initial presentation of congenital duodenal diaphragm.[ncbi.nlm.nih.gov]
  • Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The full triad of symptoms is usually not well developed in infants and children. We report a case of a 14-year-old boy with classic symptoms of pellagra.[ncbi.nlm.nih.gov]
  • The classical manifestations of pellagra include diarrhea, dementia, and dermatitis, but not all patients with pellagra present with all three manifestations.[ncbi.nlm.nih.gov]
  • We describe a case involving a patient who presented with the classic triad of "diarrhea, dermatitis, and dementia" and was promptly diagnosed and appropriately treated.[ncbi.nlm.nih.gov]
  • Pellagra is a disorder characterized by dermatitis, diarrhea, dementia and eventually death, resulting from a deficiency of niacin or its precursor tryptophan.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Early symptoms include: Loss of appetite Generalised weakness Irritability and aggression Abdominal pain Vomiting Continued deficiency leads to epithelial changes leading to: Stomatitis. Bright red glossitis. Vaginitis. Oesophagitis.[patient.info]
  • Diarrhea with nausea, vomiting, abdominal pain. . Dementia (memory disorder, confusion, disorientation etc). . Hallucination. . Fatigue, dizziness. . Insomnia (loss of sleep). . Glossitis (inflammation of tongue). .[specialityclinic.com]
  • pain, nausea and vomiting are common It may be difficult to eat and drink, leading to further malnutrition Neurological Initially symptoms of apathy and slight depression may go unnoticed Other symptoms include headache, confusion, irritability, restlessness[dermnetnz.org]
Loss of Appetite
  • ‘Inadequate amounts of this vitamin cause a disease called pellagra, which is characterized by skin disorders, weak muscles, diarrhea, and loss of appetite.’[en.oxforddictionaries.com]
  • First headaches and loss of appetite. Then pellagra struck seven of the 11 volunteers. Goldberger returned them all to the more healthful, balanced diet. The seven diseased prisoners recovered. Something in the corn was killing people. Kind of.[nowiknow.com]
  • Early symptoms include: Loss of appetite Generalised weakness Irritability and aggression Abdominal pain Vomiting Continued deficiency leads to epithelial changes leading to: Stomatitis. Bright red glossitis. Vaginitis. Oesophagitis.[patient.info]
Constipation
  • Once you recover you can maintain your health, barring some future stress/trauma that depletes your reserves, at which time heartburn/GERD/IBS/diarrhea/constipation symptoms will return, and you will lose your ability to burp easily again.[celiac.com]
  • Other side effects include nausea, vomiting, pruritus, hives, abnormally high liver enzymes, and constipation. However, too much nicotinic acid or niacin can be harmful. Avoid taking more than your doctor prescribes or recommends.[webmd.com]
  • Glossitis, estomatitis and gastrointestinal symptoms (diarrhoea, constipation or vomiting) can be associated.[doi.org]
Abdominal Cramps
  • Some Of The Symptoms Of Pellagra Are: Abdominal cramps Delusional thoughts Severe depression Diarrhea Cognitive problems Headaches Appetite loss Severe fatigue Persistent nausea that may be accompanied by vomiting Scaly skin lesions Weakness.[epainassist.com]
Cheilitis
  • Cellulitis of lips Cheilitis NOS Cheilodynia Cheilosis Exfoliative cheilitis Fistula of lips Glandular cheilitis Hypertrophy of lips Perlèche NEC Type 1 Excludes ariboflavinosis ( E53.0 ) cheilitis due to radiation-related disorders ( L55-L59 ) congenital[icd10data.com]
  • ., and Nico Mousdicas, M.D. 9 Citing Articles Three months after undergoing Roux-en-Y bariatric surgery, a 32-year-old woman presented with glossitis, angular cheilitis, and an erythematous, desquamative dermatitis with a distribution indicative of photosensitivity[nejm.org]
  • MUCOSAL INVOLVEMENT ANGULAR STOMATITIS GLOSSITIS CHEILITIS ULCERATIONS (BUCCAL / VULVAL) 15. GI MANIFESTATIONS • Precedence • • • • Nausea / Vomiting Anorexia Diarrhoea Pain Abdo 16.[slideshare.net]
Tongue Ulcer
  • Ulceration (traumatic) of tongue Type 1 Excludes atrophic glossitis ( K14.4 ) pellagrous E52 Niacin E52 (amide) Nicotinic acid deficiency E52 Pellagra E52 (alcoholic) (with polyneuropathy) Polioencephalopathy, superior hemorrhagic E51.2 ICD-10-CM Diagnosis[icd10data.com]
Sore Mouth
  • Abstract In the first few years of the present century there suddenly appeared in the Alabama Insane Hospital a new disease of unknown nature, the salient characteristics of which were a violent dermatitis of the exposed parts, sore mouth, profuse diarrhea[annals.org]
  • Locations of involvement include dorsal hands especially, as well as other photodistributed areas; frictional areas like perineum and inframammary folds; oral/ gastrointestinal with sore mouth, red tongue, flat papillae on tongue and gastrointestinal[clinicaladvisor.com]
Bleeding Gums
  • FEX: Use of case definitions and awareness of micronutrient deficiencies Summary of Unpublished Study* Afgani boy with swollen and bleeding gums typical of scurvy.[ennonline.net]
Hyperpigmentation
  • ., erythema, hyperpigmentation, and scales around the neck) (Panel B).[nejm.org]
  • After 3 months of supplementation, only mild postinflammatory hyperpigmentation remained, subjective skin sensation had returned, mood had improved and diarrhoea had abated.[mja.com.au]
  • Beneficial effects of topically applied nicotinamide in aging skin, such as improvement in barrier functions in atopic dry skin and decreased appearance of signs of facial photoaging (eg, skin texture changes and hyperpigmentation), have been noted.[emedicine.medscape.com]
Cutaneous Manifestation
  • The most common features of pellagra in patients with anorexia nervosa are cutaneous manifestations such as erythema on sun-exposed areas, glossitis, and stomatitis.[ncbi.nlm.nih.gov]
  • CUTANEOUS MANIFESTATIONS • Erythematous, Pruritic, Painful Photosensitive dermatitis • Edematous, Vesiculobullous lesions • Hyperkeratotic, hyperpigmented, sharply demarcated plaques • Painful fissured palms, soles with goose-flesh 10. • aaa 11. • Butterfly[slideshare.net]
  • The face, the neck and the dorsa of the hands are the preferential locations. 2 The skin lesions are not always found, and cases of Pellagra Sine Pellagra have been described. 3 The extra-cutaneous manifestations are less specific, but their association[bjmp.org]
Chronic Dermatitis
  • Thus, a history of these procedures should encourage the performance of a thorough history and physical exam looking for findings of pellagra or other nutritional deficiencies, especially in patients with a history of chronic dermatitis.[clinicaladvisor.com]
Visual Hallucination
  • A historical and conceptual review of the psychopathological aspects of this condition is offered, followed by the report of a patient with a history of chronic alcohol consumption showing signs of pellagra, delusions and visual hallucinations, which[ncbi.nlm.nih.gov]
  • In this case the patient presented fantastic, mystic and megalomaniac delusions and visual hallucinations.[doi.org]
  • Cognitive symptoms included mental confusion, short-term memory loss, and visual hallucinations of parasites crawling out of his skin.[consultant360.com]
Psychiatric Manifestation
  • Psychiatric manifestations are fairly common but are easily overlooked due to their non specific nature. These are commonly seen as irritability, poor concentration, anxiety, fatigue, restlessness, apathy and depression.[ncbi.nlm.nih.gov]
Confusion
  • The patient showed no evidence of mental confusion. A clinical diagnosis of pellagra was made based on the morphology, and treatment with nicotinamide 500 mg daily was instituted. The eruption quickly improved and resolved in 2 weeks (Fig. 2).[ncbi.nlm.nih.gov]
  • The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus.[ncbi.nlm.nih.gov]
  • Results and factors identified in Llopis's analysis (75 female patients) Loadings Factor 1 ‘Stupor-confusion’ (after rotation) variance 23% Emotionalism 0.644 Depression 0.710 Confusion 0.600 Delusions 0.800 Stupor 0.724 Factor 2 ‘Hallucinatory-confused[doi.org]
  • Neuropsychologic manifestation included photophobia, asthenia, depression, hallucinations, confusions, memory loss and psychosis. As pellagra advances, patient become disoriented, confused and delirious; then stuporous and finally die.[ncbi.nlm.nih.gov]
Insomnia
  • A few weeks later, the patient developed nervousness, irritability, insomnia and, consequently, delusional ideas and hallucinations, for which he had to be hospitalized.[ncbi.nlm.nih.gov]
  • Initial mental changes appear as inability to sleep (insomnia), fatigue, and a sense of disconnected-ness (apathy).[medical-dictionary.thefreedictionary.com]
  • The brain starts to degenerate causing the person to have insomnia, anxiety, aggression, and depression. Often the persons stomach bleeds, meaning they can not eat normal food and can often digest only blood.[untamedscience.com]
  • Insomnia (loss of sleep). . Glossitis (inflammation of tongue). . Stomatitis (inflammation of mucous lining of the mouth). . Vaginitis (inflammation of mucous lining of the vagina) in females. . Anemia. Dietary management for Pellagra Avoid: .[specialityclinic.com]
  • It can also present with sensitivity to sunlight (which brings on related dermatitis), aggression, skin lesions , insomnia , muscle weakness, confusion and ataxia .[house.wikia.com]
Apathy
  • At the same time, there was noticeable improvement in the patient's depression and apathy. We suggest that all of these changes could be due to percutaneous absorption of niacinamide.[ncbi.nlm.nih.gov]
  • These are commonly seen as irritability, poor concentration, anxiety, fatigue, restlessness, apathy and depression.[ncbi.nlm.nih.gov]
  • The World's Healthiest Foods Deficiency results in a condition called pellagra , the symptoms of which are a dark, scaly skin rash, a bright red tongue, vomiting, diarrhea, apathy, depression, disorientation, and memory loss.[wordnik.com]
  • The dementia , or mental aberrations , may include general nervousness, confusion, depression, apathy , and delirium. Left untreated, pellagra can lead to multiple organ failure and death.[britannica.com]
Irritability
  • A few weeks later, the patient developed nervousness, irritability, insomnia and, consequently, delusional ideas and hallucinations, for which he had to be hospitalized.[ncbi.nlm.nih.gov]
  • These are commonly seen as irritability, poor concentration, anxiety, fatigue, restlessness, apathy and depression.[ncbi.nlm.nih.gov]
  • It famously afflicted sailors exploring the New World, and its symptoms include fatigue; irritability; depression; and bleeding from the gums, under the skin, and around bones and vital organs.[pbslearningmedia.org]
  • […] appetite, abdominal pain, nausea and vomiting are common It may be difficult to eat and drink, leading to further malnutrition Neurological Initially symptoms of apathy and slight depression may go unnoticed Other symptoms include headache, confusion, irritability[dermnetnz.org]
Tremor
  • […] vomiting are common It may be difficult to eat and drink, leading to further malnutrition Neurological Initially symptoms of apathy and slight depression may go unnoticed Other symptoms include headache, confusion, irritability, restlessness, anxiety, tremor[dermnetnz.org]
  • PELLAGRA SYMPTOMS Other symptoms of Pellagra include a high sensitivity when exposed to sunlight; irritability and aggression; red skin lesions and rashes; mouth ulcers; poor appetite, nausea and vomiting; restlessness, anxiety and insomnia; tremors ,[medicalook.com]

Workup

In addition to obtaining a dietary history, low levels of urinary excretion of N-methyl nicotinamide and pyridone can be used to diagnose niacin deficiency and support the diagnosis of pellagra.

Treatment

Treatment of pellagra consists primarily of exogenous replacement of niacin or nicotinamide. However, patients with pellagra are frequently deficient in other B vitamins too and are generally prescribed B vitamin complexes. Prescription of a balanced diet and replenishment of calories is also frequently required due to the fact that pellagra patients are generally malnourished.

The dermatitis component can be treated with emolients and topical corticosteroids. Treatment of concurrent conditions such as psychiatric disorders and malabsorption syndromes should also be undertaken.

Prognosis

Prognosis of pellagra is generally good if recognized and treated early with niacin supplementation. The dermatological and diarrheal symptoms of pellagra subside over a period of 2 weeks and the neurologic symptoms following thereafter. Untreated pellagra can result in death however, due to cardiomyopathy resulting from impairment of cellular metabolic functions.

Etiology

Pellagra is caused by niacin deficiency. Niacin deficiency may occur as a result of poor dietary intake, as seen in areas corn is a staple food. Corn is deficient in niacin. However, niacin deficiency can occur as a result of deficiency in intake of its precursor molecule tryptophan. Hence, a diet poor in amino acid balance can also result in pellagra. Medical conditions in which a poor diet is a usual component, particularly anorexia nervosa and alcoholism, can manifest in pellagra.

Following on from this, excess metabolic demand for tryptophan can also cause pellagra as seen in carcinoid syndrome. Carcinoid syndrome, which is caused by excess serotonin production, diverts tryptophan towards the production of serotonin. Malabsorption disorders can also lead to pellagra due to inadequate absorption of amino acids. Hartnup disease, which is a congenital disorder of amino acid absorption, can also result in pellagra due to deficiency in tryptophan absorption.

Certain medications such as 5-fluoro-uracil, isoniazid, pyrazinamide, 6-mercaptopurine, hydantoins, phenobarbital and chloramphenicol can also induce pellagra. These medications inhibit the biosynthesis of niacin by inhibiting the conversion of tryptophan to niacin.

Epidemiology

Pellagra afflicts young adults commonly and affects both males and females equally. Historically, rural areas of the United States with high levels of poverty saw epidemic proportions of pellagra during the depression era. Although Central American cultures depend on corn as a staple, pellagra does not occur in Central America due to differences in preparation methods of the corn. Fortification of bread and flour with niacin occurred in 1941 making pellagra a rare entity in modern times. Nonetheless, pellagra remains a common entity in developing countries.

Sex distribution
Age distribution

Pathophysiology

Niacin is the precursor for nicotinamide which in turn is the precursor for nicotinamide adenine dinucleotiode (NAD). NAD in its oxidized and reduced form are known as NAD+ and NADH respectively. These two molecules play a key role in electron transfer reactions such as oxidative phosphorylation. Hence a deficiency in niacin has widespread consequences on whole body cell metabolism thereby underlying the widespread systemic symptoms pellagra. The skin photosensitivity seen in pellagra is possibly due to an indirectly induced zinc deficiency. The depression seen in pellagra may be a result of serotonin deficiency.

Prevention

Prevention of pellagra primarily depends on consumption of a balanced diet. Since 1941, flour and bread have been fortified in the United States with niacin which helped to virtually eliminate dietary-related pellagra in the United States. Primary prevention efforts are especially important in those who are at risk for poor nutrition – e.g. schizophrenics, anorexics, patients with Crohn disease or short gut syndrome. Such patients would benefit from a focus on dietary counseling and education.

Summary

Pellagra was initially described by Don Gaspar Casal in 1735 in Spanish peasants. The word "pellagra" is derived from the Latin words "pelle" - skin and "agra" - rough. Thereby describing one of the three cardinal symptoms of pellagra i.e. dermatitis, which is the most easily recognizable outward symptom of the disease. Historically, pellagra was initially recognized as caused by poor nutrition seen in poor populations living in regions where corn is the staple food. More recently, pellagra is recognized as a complication of poor nutrition secondary to other medical conditions such as alcoholism, eating disorders and schizophrenia.

Patient Information

  • Definition: Pellagra is a disease caused by niacin deficiency or tryptophan (an amino acid) deficiency. Niacin is a type of vitamin B which is important for normal metabolism. 
  • Cause: Pellagra can be caused by any condition which causes poor dietary intake, particularly of vitamins and protein. Eating as corn as a staple diet classically caused pellagra, but nowadays pellagra can occur in association with eating disorders, alcoholism, gastrointestinal diseases and other conditions where nutrition is poor.
  • Symptoms: Pellagra commonly presents with three symptoms: rash, diarrhea and mental changes.
  • Diagnosis: Pellagra is generally diagnosed clinically by asking about the symptoms of pellagra and a dietary history. Physical exam will focus on looking for the rash. A urine test may be ordered to help support the diagnosis.
  • Treatment and follow-up: Pellagra is treated with niacin supplementation as well improvement in nutrition levels overall. Dietary counseling and education is important to prevent recurrences.

References

Article

  1. Mothukuri JM, Chennamsetty SK, Bhogaraju A. Pellagra in patient with schizophrenia: a case report and review of literature. AP J Psychol Med. 2013; 14 (1):50-2.
  2. Rajakumar K. Pellagra in the United States: A Historical Perspective. Southern Medical Journal. 2000; Vol 93, No. 3.
  3. Badawy A. Alcohol and alcoholism. 2014; Vol 29, Issue 3, Pp. 238-250.
  4. Pitche PT. Sante. 2005; Jul-Sep;15(3):205-8.
  5. Hegyi J, Schwartz RA, Hegyi V. Pellagra: dermatitis, dementia and diarrhea. Int J Dermatol. 2004; Jan;43(1):1-5.
  6. Hendricks WM. Pellagra and pellagralike dermatoses: etiology, differential diagnosis, dermatopathology, and treatment. Semin Dermatol. 1991; Dec;10(4):282-92.
  7. Prinzo ZW. Pellagra and its prevention and control in major emergencies. World Health Organization 2000. WHO/NHD/00.10.
  8. Wan P, Moat S, Anstey A. Pellagra: a review with emphasis on photosensitivity. BJD 2011. 164, pp1188-1200.
  9. Prousky JE. Pellagra May Be a Rare Secondary Complication of Anorexia Nervosa: A Systematic Review of the Literature. Altern Med Rev 2003; 8(2):180-185.
  10. Oldham MA, Ivkovic A. Pellagrous encephalopathy presenting as alcohol withdrawal delirium: A case series and literature review. Addiction Science & Clinical Practice 2012; 7:12. 

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Last updated: 2017-08-09 18:12