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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