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Pellagra by aseem
- 1. PELLAGRA
- 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. 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. 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. HARTNUP DISEASE • Brush-border system defect • Defective transport / absorption of AAs CHRONIC ALCOHOLISM ANOREXIA NERVOSA FOOD FADISM FOOD ALLERGIES
- 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. CLINICAL FEATURES • 04 Ds Dermatitis Diarrhoea Dementia Death (due to MODS)
- 9. CUTANEOUS MANIFESTATIONS • Erythematous, Pruritic, Painful Photosensitive dermatitis • Edematous, Vesiculobullous lesions • Hyperkeratotic, hyperpigmented, sharply demarcated plaques • Painful fissured palms, soles with goose-flesh
- 11. • aaa
- 12. • Butterfly Rash over face
- 13. • Dorsum of Hands affected – Radial > Ulnar GAUNTLET SIGN
- 14. • Upper - portion of Neck and Chest CASAL’S NECKLACE with Sternal CRAVAT
- 15. MUCOSAL INVOLVEMENT ANGULAR STOMATITIS GLOSSITIS CHEILITIS ULCERATIONS (BUCCAL / VULVAL)
- 16. GI MANIFESTATIONS • Precedence • • • • Nausea / Vomiting Anorexia Diarrhoea Pain Abdo
- 18. DDx • • • • • • • • • • • PMLE ATOPIC DERMATITIS DRUG RASH SCLE PCT KAVA DERMOPATHY (Piper Methysticus) SEBORRHEIC DERMATITIS CONTACT DERMATITIS HYDROA VACCINIFORME PHYTOPHOTODERMATITIS ACTINIC LP
- 19. DIAGNOSIS • CLINICAL • THERAPEUTIC • Lab estimation of N-Methyl Nicotinamide • HPE
- 20. HISTOPATHOLOGY • HYPERKERATOSIS – PARAKERATOSIS • ACANTHOSIS • BALLOONING OF KERATINOCYTES / SCATTERED NECROTIC CELLS • DILATED BLOOD VESSELS • MINIMAL INFLAMM INFILTRATE
- 21. • SS
- 22. 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
- 23. 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
- 24. THANK YOU