PELLAGRA
INTRODUCTION
• Ita : Pelle – Skin ; Agra – Sour
• 1755 Françoise Thiery Vulgo Pelagrain
• 1762 – Gasper Casal in Oviedo, A...
DEFINITION
• Cellular deficiency of niacin, resulting from an
inadequate dietary supply of Niacin (Vit B3 /
Vit PP / Nicot...
ETIOLOGY
PRIMARY / DIETARY
Corn / Maize-diets (Bound Niacin)
Jowar (High Leucine content)
Uncommon in Mexicans (Nixtamaliz...
HARTNUP DISEASE
• Brush-border system defect
• Defective transport / absorption of AAs
CHRONIC ALCOHOLISM
ANOREXIA NERVOSA...
DRUGS
ISONIAZID
FLUOROURACIL
6-MP
PHENYTOIN
AZATHIOPRINE
SULFONAMIDES
ANTIDEPRESSANTS

? HIV
PATHOGENESIS
• Component of Co-I (NAD) & Co-II (NADP) that
donate or accept H+ in Carbohydrate / Fat /
Protein Metab

• Do...
CLINICAL FEATURES
• 04 Ds
Dermatitis
Diarrhoea
Dementia
Death (due to MODS)
CUTANEOUS MANIFESTATIONS
• Erythematous, Pruritic, Painful Photosensitive
dermatitis
• Edematous, Vesiculobullous lesions
...
• aaa
• Butterfly Rash over face
• Dorsum of Hands affected – Radial > Ulnar
GAUNTLET SIGN
• Upper - portion of Neck and Chest
CASAL’S NECKLACE with Sternal CRAVAT
MUCOSAL INVOLVEMENT

ANGULAR STOMATITIS
GLOSSITIS
CHEILITIS
ULCERATIONS (BUCCAL / VULVAL)
GI MANIFESTATIONS
• Precedence
•
•
•
•

Nausea / Vomiting
Anorexia
Diarrhoea
Pain Abdo
NEUROLOGICAL INVOLVEMENT
•
•
•
•
•
•
•
•

Insomnia
Fatigue
Nervousness
Apathy
Impaired Memory
Depression
Psychosis
Dementi...
DDx
•
•
•
•
•
•
•
•
•
•
•

PMLE
ATOPIC DERMATITIS
DRUG RASH
SCLE
PCT
KAVA DERMOPATHY (Piper Methysticus)
SEBORRHEIC DERMAT...
DIAGNOSIS
• CLINICAL
• THERAPEUTIC
• Lab estimation of N-Methyl Nicotinamide

• HPE
HISTOPATHOLOGY
• HYPERKERATOSIS – PARAKERATOSIS
• ACANTHOSIS
• BALLOONING OF KERATINOCYTES /
SCATTERED NECROTIC CELLS
• DI...
• SS
TREATMENT (DIETARY)
• TRYPTOPHAN-RICH FOODS viz Liver / Eggs / Legumes / Nuts
• RDA (NIACIN)
Males: 19-50 years : 19 mg/da...
TREATMENT (MEDICAL)
• NICOTINAMIDE
500mg / day X 03-04 weeks
Neurological reversal within 24-48 hrs
Cutaneous resolution w...
THANK YOU
Pellagra by aseem
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Pellagra by aseem

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Pellagra by aseem

  1. 1. PELLAGRA
  2. 2. INTRODUCTION • Ita : Pelle – Skin ; Agra – Sour • 1755 Françoise Thiery Vulgo Pelagrain • 1762 – Gasper Casal in Oviedo, Asturias ; poor peasants – Mal de la rose • 1771 - Francesco Frapolli - ‘PELLAGRA’ • 1902 - American epidemic • Goldberger (1926) – Nutritional correction reversal
  3. 3. DEFINITION • Cellular deficiency of niacin, resulting from an inadequate dietary supply of Niacin (Vit B3 / Vit PP / Nicotinic Acid) and tryptophan, or high Leucine, is termed Pellagra •
  4. 4. ETIOLOGY PRIMARY / DIETARY Corn / Maize-diets (Bound Niacin) Jowar (High Leucine content) Uncommon in Mexicans (Nixtamalization) GI DISEASE Jejuno-Ileitis / Prolonged Diarrhoea / IBD MALABSORPTION SYNDROME CARCINOID SYNDROME
  5. 5. HARTNUP DISEASE • Brush-border system defect • Defective transport / absorption of AAs CHRONIC ALCOHOLISM ANOREXIA NERVOSA FOOD FADISM FOOD ALLERGIES
  6. 6. DRUGS ISONIAZID FLUOROURACIL 6-MP PHENYTOIN AZATHIOPRINE SULFONAMIDES ANTIDEPRESSANTS ? HIV
  7. 7. PATHOGENESIS • Component of Co-I (NAD) & Co-II (NADP) that donate or accept H+ in Carbohydrate / Fat / Protein Metab • Downregulates pro- inflamm CKs (IL-1,6,8,12 / TNF-A) • Produces Ceramides / Corneal Lipids – maintain Epithelial Permeability, Decrease Basal Cell Proliferation
  8. 8. CLINICAL FEATURES • 04 Ds Dermatitis Diarrhoea Dementia Death (due to MODS)
  9. 9. CUTANEOUS MANIFESTATIONS • Erythematous, Pruritic, Painful Photosensitive dermatitis • Edematous, Vesiculobullous lesions • Hyperkeratotic, hyperpigmented, sharply demarcated plaques • Painful fissured palms, soles with goose-flesh
  10. 10. • aaa
  11. 11. • Butterfly Rash over face
  12. 12. • Dorsum of Hands affected – Radial > Ulnar GAUNTLET SIGN
  13. 13. • Upper - portion of Neck and Chest CASAL’S NECKLACE with Sternal CRAVAT
  14. 14. MUCOSAL INVOLVEMENT ANGULAR STOMATITIS GLOSSITIS CHEILITIS ULCERATIONS (BUCCAL / VULVAL)
  15. 15. GI MANIFESTATIONS • Precedence • • • • Nausea / Vomiting Anorexia Diarrhoea Pain Abdo
  16. 16. NEUROLOGICAL INVOLVEMENT • • • • • • • • Insomnia Fatigue Nervousness Apathy Impaired Memory Depression Psychosis Dementia
  17. 17. DDx • • • • • • • • • • • PMLE ATOPIC DERMATITIS DRUG RASH SCLE PCT KAVA DERMOPATHY (Piper Methysticus) SEBORRHEIC DERMATITIS CONTACT DERMATITIS HYDROA VACCINIFORME PHYTOPHOTODERMATITIS ACTINIC LP
  18. 18. DIAGNOSIS • CLINICAL • THERAPEUTIC • Lab estimation of N-Methyl Nicotinamide • HPE
  19. 19. HISTOPATHOLOGY • HYPERKERATOSIS – PARAKERATOSIS • ACANTHOSIS • BALLOONING OF KERATINOCYTES / SCATTERED NECROTIC CELLS • DILATED BLOOD VESSELS • MINIMAL INFLAMM INFILTRATE
  20. 20. • SS
  21. 21. TREATMENT (DIETARY) • TRYPTOPHAN-RICH FOODS viz Liver / Eggs / Legumes / Nuts • RDA (NIACIN) Males: 19-50 years : 19 mg/day; >50 years : 15 mg/day Females: 19-50 years : 15 mg/day; >50 years : 13 mg/day Pregnant: 18 mg/day Nursing: 17 mg/day • NIXTAMALIZED MAIZE (CURED CORN) • AVOIDANCE OF JOWAR
  22. 22. TREATMENT (MEDICAL) • NICOTINAMIDE 500mg / day X 03-04 weeks Neurological reversal within 24-48 hrs Cutaneous resolution within 04 weeks • NIACIN 50-100mg PO TDS X 03-04 weeks • TREATING UNDERLYING CAUSES IN SECONDARY PELLAGRA
  23. 23. THANK YOU

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