pellagra by aseem
DESCRIPTION
TRANSCRIPT
PELLAGRA
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
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
•
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
HARTNUP DISEASE• Brush-border system defect• Defective transport / absorption of AAs
CHRONIC ALCOHOLISM
ANOREXIA NERVOSA
FOOD FADISM
FOOD ALLERGIES
DRUGSISONIAZIDFLUOROURACIL6-MPPHENYTOINAZATHIOPRINESULFONAMIDESANTIDEPRESSANTS
? HIV
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
CLINICAL FEATURES
• 04 Ds
DermatitisDiarrhoeaDementiaDeath (due to MODS)
CUTANEOUS MANIFESTATIONS
• Erythematous, Pruritic, Painful Photosensitive dermatitis
• Edematous, Vesiculobullous lesions
• Hyperkeratotic, hyperpigmented, sharply demarcated plaques
• Painful fissured palms, soles with goose-flesh
• aaa
• Butterfly Rash over face
• Dorsum of Hands affected – Radial > Ulnar GAUNTLET SIGN
• Upper - portion of Neck and ChestCASAL’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• Dementia
DDx
• PMLE• ATOPIC DERMATITIS• DRUG RASH• SCLE• PCT• KAVA DERMOPATHY (Piper Methysticus)• SEBORRHEIC DERMATITIS• CONTACT DERMATITIS• HYDROA VACCINIFORME• PHYTOPHOTODERMATITIS• ACTINIC LP
DIAGNOSIS
• CLINICAL
• THERAPEUTIC
• Lab estimation of N-Methyl Nicotinamide
• HPE
HISTOPATHOLOGY
• HYPERKERATOSIS – PARAKERATOSIS• ACANTHOSIS• BALLOONING OF KERATINOCYTES /
SCATTERED NECROTIC CELLS• DILATED BLOOD VESSELS• MINIMAL INFLAMM INFILTRATE
• SS
TREATMENT (DIETARY)
• TRYPTOPHAN-RICH FOODS viz Liver / Eggs / Legumes / Nuts
• RDA (NIACIN)Males: 19-50 years : 19 mg/day; >50 years : 15 mg/dayFemales: 19-50 years : 15 mg/day; >50 years : 13 mg/dayPregnant: 18 mg/dayNursing: 17 mg/day
• NIXTAMALIZED MAIZE (CURED CORN)
• AVOIDANCE OF JOWAR
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
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