Pellagra E52

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.12.2020

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Synonym(s)

vitamin B3 deficiency; Vitamin B3 deficiency; Vitamin B3 Hypovitminosis; vitamin B6 deficiency

History
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Casál, 1762

Definition
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Rare avitaminosis due to lack of vitamin B3 and possibly other factors of the B-complex (vitamin B6) with skin, intestinal and CNS disorders. Pellagra occurs when the diet consists mainly of maize or sorghum millet. The bound form of niacin (niacytin) present there cannot be utilised by the body. Thus, the disease was widespread in poor regions of Southern Europe and America before the connections were known. Vitamin B3 is mainly found in meat, fish, milk, eggs, rice and minerals. The daily requirement of vitamin B3 is 5-20mg.

Occurrence/Epidemiology
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Endemic with preferred corn nutrition, especially in Italy, Balkans, southern states of the USA, Mexico.

In malnutrition (alcoholism), malabsorption and permanent intake of drugs ( isoniazid, hydantoins, azathioprine, etc.).

Manifestation
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In older writings, the maximum of the disease is given between the 30th-50th year of life (see also illustrations from 1908).

Time of manifestations (time of the spring equinoxes) about the end of March.

In Europe, vitamin B3/B6 deficiency tends to occur in people aged >50 years.

Localization
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Especially uncovered (UV-irradiated) parts of the body: back of fingers and hands, shin, face, chest, neck, neck (S.a. Casal's collar).

Clinical features
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  • Acute "photoprovoked" dermatitis, combined with changes in the intestinal tract and nervous system, characterized by 4 "D": diarrhea, dermatitis, dementia, death.
  • Notice! Often the internal or neurological symptoms are at the forefront of the disease!

  • Skin symptoms: Mostly symmetrical, large-area (5.0-10.0 cm), sharply defined, initially red, later reddish-brown, spots that turn into plaques and fine-lamellar scaling. Blistering or gangrenous ulcerations are possible but rather rare. Typical is the seasonal nature of the disease! Triggered by sunlight and mechanical stress. In case of longer duration: thickening of the skin, painful fissures or rhagades, diffuse palmareratoses.
  • Typical (if not treated) is a recurrent course of symptoms with increasing atrophy of the lesional skin.
  • A generalized livedo reticularis has also been described
  • Mucous membrane changes: Stomatitis, glossitis, vulvitis, dry, chapped, reddened lips. Severe sialorrhea.
  • Gastrointestinal problems: abdominal pain, nausea, vomiting, diarrhoea, in 50% of cases reduced hydrochloric acid production in the stomach.
  • Neurological phenomena: depression, apathy, peripheral polyneuritis, myelitis, psychosis.
  • Pellagroid, pyridoxine deficiency, pernicious anemia, avitaminosis-folic acid, pantothenic acid deficiency.

Laboratory
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Measurement of the vitamin B3 level (methyl nicotinamide) in urine (using HPLC = high pressure liquid chromatography)

  • Standard value: 1600-4300ug/gCratinine
  • Malnourished: 500ug/g creatinine (pellagra)

Differential diagnosis
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The most important differential diagnosis is a phototoxic or photoallergic dermatitis! Skin changes occur mainly in the area of the exposed areas! Time of manifestations. Here the neurological conspicuity is missing

Furthermore:

  • Photoxic dermatitis: medical history, no diarrhea, no neurological changes
  • Polymorphic light dermatosis: variable course with known pattern, no diarrhoea, no neurological changes, no hyperpigmentation
  • Porphyria cutanea tarda: blister formation after banal mechanical irritation, dark urine! Hepatopathy
  • Porphyria variegata: erythema, blisters, colicky abdominal pain, porphyrin detection in stool and urine
  • Drug reactions: anamnesis, clinical course
  • Acrodermatitis enteropathica: acral localization of the eczematous changes; no sunlight-exposed areas, no hyperpigmentation
  • Kwashiorkor: generalized edema, protein deficiency, thin light hair

Therapy
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Nicotinamide (e.g. Nicobion Tbl.) in therapeutic dosage, e.g. 100-300 mg/day p.o. Intravenous substitution only in very severe cases (50-250 mg/day), high protein diet. As prophylaxis: 15-30 mg/day p.o.

Note(s)
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Name: derived from "Pelle agra" (= rough skin).

Literature
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  1. Badawy AA (2014) Pellagra and alcoholism: a biochemical perspective. Alcohol Alcohol 49: 238-250

  2. Hegyi J et al (2004) Pellagra: dermatitis, dementia, and diarrhea. Int J Dermatol 43: 1-5

  3. Hendricks WM (1991) Pellagra and pellagralike dermatoses: etiology, differential diagnosis, dermatopathology and treatment. Semin Dermatol 10: 282-292
  4. Karthikeyan K et al (2002) Pellagra and skin. Int J Dermatol 41: 476-481
  5. Merk L (1908) The skin manifestations of pellagra. Published by Julius Springer, Berlin, plates V,VI, VIII and XIII.
  6. Major RH (1944) Don Gasper Casal, Francois Thiery and pellagra. Bull Hist Med 16: 351-361
  7. Montag A (2016) Pellagra yesterday and today- On the trail of a century-old mystery. Act Dermatol 42: 131-138
  8. Pfeiffer RF (2014) Neurologic manifestations of malabsorption syndromes. Handb Clin Neurol 120:621-632

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 18.12.2020