Pellagra is a systemic disease caused by niacin (vitamin B3) deficiency. It is characterized by 4 D's: dermatitis, diarrhea, dementia, and death if left untreated. It results from a diet lacking in niacin and tryptophan, which is converted to niacin in the body. Historically, it was common where maize and rice were staples since processing removes the niacin precursor tryptophan. Symptoms include scaly skin rashes, especially on sun-exposed areas, inflammation of the mouth and tongue, diarrhea, and progressive dementia. Treatment involves niacin supplementation either orally or via IV.
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1. Balakrishnan D
BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Pellagra
A systemic disease, due to Vitamin deficiency
(Deficiency of Vitamin B 3, Niacin, Nicotinamide)
BSc MBBS MS DLO PhD
3. BAS17201 Neurology
Balakrishnan D 20200427
Dermatitis
Diarrhoea
Dementia
3 D
Pellagra
If not treated promptly, a fourth D occurs: DEATH
4. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
A systemic disease, due to Vitamin deficiency
Vitamin B3, Niacin, Nicotinamide
Pellagra is a consequence of a diet, low in both Niacin B3, and the amino acid Tryptophan.
Tryptophan is a precursor for the vitamin B3.
Pellagra reverts to normal, within days of oral supplementation.
Pellagra
5. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Pellagra used to be common in Africa and India, where the staple diet is Rice / Corn / Maize.
In US, Pellagra was prevalent in the south, in early 1900s, when corn was consumed largely.
The earliest description of pellagra was by a Spanish physician Don Gaspar Casal, in 1763.
He described all the clinical features. He correctly ascribed the disease to unbalanced diets
(based on maize), of the poor peasants in the Asturia region of Spain.
Presently, this condition has been almost wiped out by better nutrition, consumption of milk
products, fortification of bread etc. But, it still lingers in Refugee / Returnee populations.
Pellagra
6. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Dementia, Delusions or mental confusion.
Diarrhoea, Pain in abdomen, loss of appetite, weakness.
Dermatitis forms around the neck (Casal necklace)
Scaly skin sores, especially in sun-exposed areas of the skin.
Mucositis (inflamed oral cavity, bright red tongue, angular cheilitis
Present in many alcoholics.
Clinical features
7. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Dermatitis
Red, flaky skin, with distinct margins
Areas of discoloration, ranging from red to brown
thick, crusty, scaly, or cracked skin
itchy, burning patches of skin,
Sun exposed parts e.g. Neck: Casal necklace.
Inflamed mucous membrane, bright red tongue.
9. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
In this disease, the skin peels away, resembling the Holly leaves.
Please note the distinct margins of the lesions
Pellagra
pel: leaf, agra: coarse, rough
11. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Dementia
Progressive dementia
Apprehension (fear) and confusion in the early stages.
Progressing to severe derangement with maniacal outbursts.
Confusion, Disorientation
Ataxia also is seen in some persons
Spastic para paresis, Peripheral neuropathy
In the late stages, the worst occurs,
12. BAS17201 Neurology
Balakrishnan D 20200427
In preparing Maize, Corn and Rice, after you
remove the husk, there is one more layer
called Aleurone layer. This contains certain
amino acids particularly Tryptophan. This
is a precursor to Niacin. Originally, the The
American natives used to soak maize in lime
water. Some in India used to parboil the
paddy, before milling. Both practices are not
practised now. Polishing the rice, removes
the aleurone layer.
13. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Tryptophan
Tryptophan is a precursor to
neuro transmitter, Serotonin
hormone Melatonin and
vitamin B3 (Niacin).
Tryptophan is an essential amino acid i.e. humans cannot synthesize it. It must be
obtained from the diet. It is plentiful in Chocolate, dried dates, Oats, Peanuts,
Chickpeas, Almonds
Milk, Yoghurt, curds, cheese,
Meat, fish, eggs, Poultry, Sesame, Sunflower seeds, Pumpkin Buckwheat, Spirulina
14. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Tryptophan
Tryptophan is a precursor to
neuro transmitter, Serotonin
hormone Melatonin and
vitamin B3 (Niacin).
Tryptophan is an essential amino acid i.e. humans cannot synthesize it. It must be
obtained from the diet. It is plentiful in Chocolate, dried dates, Oats, Peanuts,
Chickpeas, Almonds
Milk, Yoghurt, curds, cheese,
Meat, fish, eggs, Poultry, Sesame, Sunflower seeds, Pumpkin Buckwheat, Spirulina
Germination potentiates the presence of Niacin & Tryptophan.
15. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Depletion of niacin from food stuff by bad practices
Reduced intake due to various causes, including alcohol
Not taking enough food, containing the precursor, Tryptophan
Non availability of glucose to the body (Brain)
Brain tissue damage infarction - Neuropathy
Pathogenesis
16. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Secondary pellagra are caused by the following:
(a) malabsorption: usually associated with jejuno-ileitis, gastroenterostomy and Crohn's
disease.
(b) chronic alcoholism: pellagra in chronic alcoholics is usually due to a combination of
their diets being very limited in niacin and tryptophan, and due to an incomplete
absorption of the already poor diet.
(c) abnormalities of tryptophan metabolism: in Hartnup's disease there is a block in the
tryptophannicotinic acid pathway which can lead to pellagra due to nicotinic acid
deficiency
(d) chemotherapeutic agents: the following are known to provoke pellagra: Isoniazid,
6-Mercaptopurine, 5-Fluorouracil and Chloramphenicol.
17. BAS17201 Neurology
Balakrishnan D 20200427
Pellagra
Differential Diagnosis & investigations
All the diseases which can cause diarrhoea, dermatitis and dementia, just like
the Beriberi, Wernickes, Korsakoff, are the differential diagnosis, for this
condition. Almost, similarly, alcoholism history will be present in both the
conditions.
The laboratory investigations will have to focus on ruling out these
conditions.
Urinary excretion of Niacin
HPLC methods for estimating the Plasma levels of Nicotinamide