Nutritional Dermatoses

1. Nutritional Dermatoses

2. Stages of nutritional deficiency syndrome • Stage I Intake falls below daily requirement but the reserves maintain normal blood values • Stage II Blood levels decrease but patient is asymptomatic • Stage III Development of clinical signs and symptoms

3. Causes of deficiencies • Increased requirement • During growth • Pregnancy • Lactation • Fever • Hyperthyroidism Decreased intake • Poverty • Ignorance • Food faddism • Crash diets • Anorexia nervosa

4. Stages of nutritional deficiency syndrome Decreased absorption and utilization • GI - mucosal disturbances: Malabsorption syndrome • Dietary factors: High dietary phytate, TPN, alcoholism • Trauma: Burns , Post surgical procedures • Malignancy • Renal disorders • Infections: Parasitic, bacterial, viral • Miscellaneous: Collagen vascular disease, HIV

5. Nutritional deficiencies • Fat soluble vitamins (A,D,E,K) • Water soluble vitamins (B-complex, Niacin, Pantothenic acid, Biotin, Vit C) • Minerals • Trace elements (Zinc, Iron) • Essential fatty acids (EFA) • PEM (Protein energy malnutrition)

6. Important Points 1. Water soluble vitamins • Not stored in body • Excessive consumption No toxicity 2. Fat soluble vitamins (A, D, E, K) • Stored in liver • Excessive consumption toxicity

7. Vitamin A (Retinol) deficiency Rich source • Animal fats, fish liver oils, milk, butter, eggs, liver , kidneys Provitamin A (Beta carotene) • Green and yellow parts of plants (spinach, drum sticks, spring onions, cabbage, pumpkin, carrots, tomato) • Fruits – mango, papaya

8. Clinical features Skin manifestations: • Infants, very young children Extensive xerosis - an earliest manifestation • Adults: Follicular hyperkeratosis • Sites: extensor of extremities; spreads to shoulder, face, chest, abdomen, back , buttocks. Dry, firm brown follicular papule with keratotic plug covered with loosely adherent scale On removal of plug, a pit is seen

9. Clinical features Skin manifestations: • Phrynoderma (Toad skin) Mild follicular hyperkeratosis, limited distribution • Mixed deficiencies of Vit.A, Vit.E, B - complex, Vit.C and EFA

10. Clinical features Eye Manifestations: Common cause of blindness in developing countries • Earliest symptoms: • Nyctalopia (night blindness) • Hemeralopia (inability to see bright light) • Xerophthalmia: • Conjunctivalxerosis • Bitot’s spot • Corneal xerosis • Corneal ulceration • Keratomalacia • Phthisis bulbi, scarring, blindness

11. Treatment Normal requirements: • Infants, children < 4 yrs - 1500 IU of Vit. A > 4 yrs - 5000 IU of Vit. A Prophylactic Treatment: • Vit. A (2 lacs IU) every 6 months to pre-school children (Orally retinylpalmitate in oil) • Good quality protein diet + vitamins • Education of local community Contd…

12. Treatment • Dose of 1-3 lacs IU of Vit. A for 1-3 days (Stop in case of headaches) • Supplements: Vit. B complex and EFAs • Diet to include eggs, milk, butter, green leafy vegetables • Treatment of underlying cause (especially protein and zinc deficiency)

13. Vitamin D • Vit. D is a group of antirachitic sterol derivatives, considered as a hormone • Skin: Role in synthesis, storage and release of Vit. D

14. Source of Vit. D and Chemistry • Plants ergosterolergocalciferol (Vit D2) • Animal and dairy products (Eggs, liver, butter, codliver oil) 7 dehydrocholesterol (human skin) UVB Cholecalciferol (Vit. D3) Liver 25 hydroxycholecalciferol Kidney 1, 25 di-hydroxycholecalciferol (Calcitriol)

15. Clinical features and treatment Children: • Rickets, Tetany Adults: • Osteomalacia • Type I Vit.D dependent rickets : No skin lesions • Type II Vit.D resistant rickets : Progressive alopecia Treatment: • Normal daily requirement: 400 IU • In rickets : 5000 IU oral Vit D for 3 to 5 weeks • Exposure to sunlight

16. Vitamin B Complex • Mostly combined deficiencies occur due to insufficient proteins or other essential nutrients (Zinc, EFA)

17. Vitamin B1 (Thiamine, Aneurin) Sources • Yeast (richest source), unmilled cereals, pulses, nuts Beriberi Dry • Peripheral neurologic syndrome, atrophic skin, red burning tongue • Korsakoff'spsychosis,Wernicke's encephalopathy Beriberi Wet • High output cardiac failure Skin is warm before CCF and cold, edematous, cracked later

18. Diagnosis and treatment Diagnosis • Urinary excretion of < 50 mcg of thiamine after 1 mg injection Treatment: • Dietary requirement - 0.5 mg /1000 kcal, 0.5 to 2 mg • Beriberi - 10 to 100 mg / day • If severe - add manganese (corrects thiamine resistance) • B - complex vitamins are supplemented • Local application of zinc oxide ointment , mineral oil (for cracked skin)

19. Riboflavin deficiency (Vit. B2): (Oro - Oculo - Genital syndrome) Sources of Vit. B2: • Milk, milk products, eggs, liver, cereals, pulses, green leafy vegetables • Deficiency of Vit.B2 affects metabolism of free fatty acids, tryptophan, folic acid • Presents as overlapping manifestations

20. Clinical features • Seen after 3-5 months of inadequate diet It is also known as “pellagra sine pellagra” • Oral manifestations: • Angular stomatitis (perleche) with candidiasis • Cheilosis : lip involvement with vertical fissuring • Glossitis : magenta coloured tongue atrophic filiform papillae enlarged fungiform papillae

21. Clinical features Skin manifestations: • Seborrheic dermatitis like rash; dyssebacea Fine greasy scales with erythema over nasolabial folds, ala nasi, nasal bridge, forehead, eyelids, earlobes • Dysriboflavinosis Dyskeratotic follicular papules with scaly erythema • Patchy alopecia with scaling on scalp and eyebrows

22. Clinical features • Genitals: Earliest manifestation (scrotum, vulva) Early - Patchy redness, fine powdery desquamation Late - Lichenification Severe - Raw areas over shaft of penis, inner thighs • Eyes: Photophobia, lacrimation, blepharospasm, conjunctivitis, decrease in visual acuity, corneal vascularization • CNS: Psychomotor, intellectual development impaired in children

23. Diagnosis and treatment Diagnosis • Urinary excretion < 30 mcg of vit.B2 / gm of creatinine Treatment: • Normal requirement : 1-2 mg / day mg • Therapeutic dose: Infants : 1-3 mg Adults : 10-30 mg • Correct the associated tryptophan, FA, EFA deficiency

24. Vitamin B3 (Nicotinic acid, Nicotinamide, Niacin ) Pellagra (deficiency of Niacin) • Italian word pelle - skin, agra - rough • First described in 1735 by Casal in Spain • Niacin includes both nicotinic acid and niacinamide • Niacinamide is active form and is converted to coenzymes NAD, NADP • Plays a vital role in cell, fatty acid, carbohydrate metabolism

25. Sources • Meat, fish, eggs • Milk, cheese • Cereals, grains, legumes • Coffee and tea • Endogenous production 60 mg of tryptophan 1mg of niacin

26. Etiology • Staple diet of maize and jowar with less animal proteins • Maize - poor source of nicotinic acid and tryptophan - niacin is present but not bio-available • Jowar - high content of leucine Imbalance in leucine and isoleucine inhibition of NAD Tryptophan Niacin • Chronic alcoholics - unbalanced diet • Malabsorption

27. Clinical features • This disease is characterized by 4 “D’s” • Dermatitis • Dementia • Diarrhea • Death • Prodrome - weakness, fatigue

28. Clinical features Skin: • Photo exposed areas • Erythema - well demarcated patches with pruritus and burning, slight edema • Blisters, dry brown scales • Pressure sites, shoulder, elbow, buttocks, knee • Intertriginous area - redness, maceration

29. Clinical features • Pellagrins nose Dull erythema, butterfly rash with scaling on bridge of nose • Casal's necklace Sharply demarcated lesion on upper central chest, neck • Cravat Anterior continuation of necklace on chest • Scrotal erythema Symmetrical lesions, clear line of demarcation

30. Clinical features • Mucous membrane • Angular stomatitis, cheilitis • Scarlet glossitis with imprint of teeth • Tongue is red, smooth, atrophy of filiform papillae, erosions, ulcerations, fissures • Swelling of parotid gland, increased salivation • GIT: Anorexia, nausea, vomiting, abdominal pain, bloody diarrhoea • CNS: Depression, psychosis

31. Treatment Daily requirement - 10 to 20 mg / day Therapeutic dose: • 300 - 500 mg niacinamide orally or intramuscular in divided doses (amide preferred because it does not precipitate flushing, itching, burning) • Supplement with B complex, animal proteins eggs, milk • Balanced diet • Reduce alcohol

32. Vitamin B6 deficiency (Pyridoxine) • Animal sources: Liver, egg yolk, meat • Vegetable sources: Pulses, cereals, peas, soya beans • Pyridoxine deficiency occurs during administration of drugs like: INH Hydralazine Cycloserine Penicillamine

33. Clinical features • Children: convulsion, anemia • Adults: seborrheic dermatitis like rash, cheilitis, angular stomatitis, glossitis, peripheral neuritis • Chinese restaurant syndrome : (Inability to metabolize monosodium glutamate) Headache, sensation of pressure in chest, palpitation, feeling of warmth, tingling, numbness

34. Diagnosis and treatment Diagnosis • Serum Pyridoxal phosphate levels < 20 mcg / ml Treatment: • Daily requirement: 1.5 - 2.5 mg • Therapeutic dose: 30 -100 mg / day orally

35. Vit B12 deficiency (Cobalamin, Cyanocobalamin) • Sources: • Liver, kidney, heart - richest • Meat, fish, cheese, eggs, milk • Vegetables, fruits, legumes - nil; but present if contaminated by bacteria • Vit B12 is synthesized in colon (low bio-availability)

36. Cause of deficiency of Vit.B12 • Strict vegetarian diet • Gastric atrophy (achlorhydria) and decreased intrinsic factor (pernicious anemia) • Diphyllobothriumlatuminfestation • Malabsorption syndromes (sprue, intestinal TB, Whipple’s disease) • Elderly individuals, chronic alcoholism

37. Clinical features • Skin Symmetrical generalized hyperpigmentation (greyish - brown) • Mucous membrane Hyperpigmentation, cheilitis, glossitis with beefy red tongue, glossodynia, aphthae like lesions • Nails: Pigmented streaks • Hair: Premature graying, canities • Other manifestations: Megaloblastic, pernicious anemia, peripheral neuritis, poor memory

38. Diagnosis • Serum Vit. B12 <150 pg/ml • Hemogram • Bone marrow examination • Schilling’s test - measures radioactive Vit. B12 with and without intrinsic factor

39. Treatment • Daily requirement :1 mcg • Dose : 1000 mcg / week for 1 month; 1000 mcg / month thereafter • Also add folic acid 1- 5 mg • Course: • Cutaneous changes improve within 1 year • In pernicious anemia Vitamin B12 given life long

40. Folic acid (Vit. B9)(Pteroyl - glutamic acid, folacin) Sources: • Liver, meat, green leafy vegetables, milk • Produced by colonic bacteria (inadequate) Folic acid and Vit. B12 are interdependent, therefore the deficiencies occur simultaneously • Folic Acid Folinic acid (active form) Vit C

41. Clinical features • Skin: Diffuse hyperpigmentation • Mucous membrane: Glossitis, superficial erosions, cheilitis • Others: Megaloblastic anemia

42. Diagnosis and treatment Diagnosis Serum folate < 3 ng/ml (normal > 6 ng/ml) Treatment • Daily requirement : 50 -100 mcg • In pregnancy : 400 mcg Therapeutic dose: 1- 5 mg / day; also correct Vit. B12 deficiency

43. Vitamin C (Ascorbic acid) • Scurvy: Deficiency of Vitamin C Sources: • Fresh fruits - oranges, grapes, lemons • Fresh vegetables - Green leafy vegetables, potatoes, cabbage Functions: • Role in collagen and ground substance formation, wound healing, immune response • Required for iron absorption

44. Causes • Diet poor in Vitamin C (elderly men, alcoholics) • Gastro-intestinal diseases • Malnourished children with scurvy (Barlow's disease) • Seen in cigarette smokers

45. Clinical features Skin • Follicular hyperkeratosis : Earliest change, cork screw hair (swan neck deformity) - due to reduced disulfide bond • Perifollicular hemorrhage Sites: upper arms, buttocks, shins, trunk, thighs • Petechiae, echhymosis • “Woody” edema of legs • Delayed wound healing

46. Clinical features Oral Cavity: • Hemorrhagic gingivitis - spongy gum • Loosened teeth, foul odour Internal hemorrhage : • Hematuria, epistaxis, malena, hematemesis In infants: • Excessive crying • Pseudo paralysis • Scorbutic rosary

47. Treatment • Daily requirement Adult: 50 mg Children: 25 mg • Therapeutic dose: 100 - 300 mg / day

48. Minerals and Trace elements Zinc : • It is metal moiety of important enzymes for carbohydrate, protein, lipid and nucleic acid metabolism • Role in immunological functions and wound healing Sources: • Shellfish, legumes, nuts, whole grains, green leafy vegetables

49. Zinc deficiency Genetic • Acrodermatitisenteropathica Acquired • Acquired zinc deficiency

50. Acrodermatitisenteropathica • Transmitted as autosomal recessive trait • First described by Danbolt and Class in 1943 • Etiology: • Deficient zinc binding protein called zinc ligand binding (ZLB)