Nutritional Dermatoses 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 Causes of deficiencies Decreased intake Increased requirement Poverty During growth Ignorance Pregnancy Food faddism Lactation Crash diets Fever Anorexia nervosa Hyperthyroidism 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 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) 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 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 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 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 Clinical features Eye Manifestations: Common cause of blindness in developing countries Earliest symptoms: ◦ Nyctalopia (night blindness) ◦ Hemeralopia (inability to see bright light) Xerophthalmia: ◦ Conjunctival xerosis ◦ Bitot’s spot ◦ Corneal xerosis ◦ Corneal ulceration ◦ Keratomalacia ◦ Phthisis bulbi, scarring, blindness 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 retinyl palmitate in oil) Good quality protein diet + vitamins Education of local community Contd… 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) 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 Source of Vit. D and Chemistry Plants ergosterol ergocalciferol (Vit D2) Animal and dairy products (Eggs, liver, butter, codliver oil) 7 dehydrocholesterol (human skin) UVB Cholecalciferol (Vit. D3) Liver 25 hydroxy cholecalciferol Kidney 1, 25 di-hydroxy cholecalciferol (Calcitriol) 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 Vitamin B Complex Mostly combined deficiencies occur due to insufficient proteins or other essential nutrients (Zinc, EFA) Vitamin B1 (Thiamine, Aneurin) Sources Yeast (richest source), unmilled cereals, pulses, nuts Beriberi Dry Peripheral neurologic syndrome, atrophic skin, red burning tongue Korsakoff's psychosis,Wernicke's encephalopathy Beriberi Wet High output cardiac failure Skin is warm before CCF and cold, edematous, cracked later 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) 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 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 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 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 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 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 Sources Meat, fish, eggs Milk, cheese Cereals, grains, legumes Coffee and tea Endogenous production 60 mg of tryptophan 1mg of niacin 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 of NAD Tryptophan Niacin Chronic alcoholics - unbalanced diet Malabsorption inhibition Clinical features This disease is characterized by 4 “D’s” ◦ Dermatitis ◦ Dementia ◦ Diarrhea ◦ Death Prodrome - weakness, fatigue 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 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 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 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 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 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 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 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 bioavailability) Cause of deficiency of Vit.B12 Strict vegetarian diet Gastric atrophy (achlorhydria) and decreased intrinsic factor (pernicious anemia) Diphyllobothrium latum infestation Malabsorption syndromes (sprue, intestinal TB, Whipple’s disease) Elderly individuals, chronic alcoholism 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 Diagnosis Serum Vit. B12 <150 pg/ml Hemogram Bone marrow examination Schilling’s test - measures radioactive Vit. B12 with and without intrinsic factor 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 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 Vit C Folic Acid Folinic acid (active form) Clinical features Skin: Diffuse hyperpigmentation Mucous membrane: Glossitis, superficial erosions, cheilitis Others: Megaloblastic anemia 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 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 Causes Diet poor in Vitamin C (elderly men, alcoholics) Gastro-intestinal diseases Malnourished children with scurvy (Barlow's disease) Seen in cigarette smokers 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 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 Treatment Daily requirement Adult: 50 mg Children: 25 mg Therapeutic dose: 100 - 300 mg / day 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 Zinc deficiency Genetic Acrodermatitis enteropathica Acquired Acquired zinc deficiency Acrodermatitis enteropathica Transmitted as autosomal recessive trait First described by Danbolt and Class in 1943 Etiology: Deficient zinc binding protein called zinc ligand binding (ZLB) Clinical features Disease occurs within few weeks after birth if bottle fed or 4-6 weeks after weaning from breast milk Perlèche : early sign; angular stomatitis, glossitis Perioral and peri-orificial rash Vesiculobullous and pustular lesions Superadded infection with candida is common Nails : paronychia, white spots Hair : alopecia Eyes : photophobia, conjunctivitis Diarrhoea, growth failure, emotional and mental disturbances Diagnosis and treatment Diagnosis Serum zinc levels <80 mcg/dl (80 - 120 mcg/dl) Treatment Daily requirement: Infants < 6months : 3 mg 6months - 1yr : 5 mg 1 - 7 yrs : 10 mg > 7 yrs, adults : 16 mg Pregnant and lactating mothers : 20 -25 mg Treatment Dose Oral zinc : 2mg/kg/day for 1- 2 weeks 30 to 55 mg of elemental Zn for 1-2 weeks (220 mg ZnSo4 = 55 mg of elemental Zn) Hereditary type requires life long treatment Iron deficiency Sources: Green leafy vegetables, pulses, meat products Vitamin C rich foods improve absorption; tea and tamarind inhibits absorption Clinical features: Generalized pruritus, increased hair loss, koilonychia Angular stomatitis, cheilitis, glossitis Hypochromic microcytic anemia Plummer-Vinson syndrome Treatment Therapeutic dose: Ferrous sulphate or gluconate 300mg thrice daily Treat underlying cause: chronic blood loss, parasitic infestations, malaria Supplementation with Vitamin C Supplementation during pregnancy Protein Energy Malnutrition (PEM) PEM is most common form of malnutrition Age: 1-3 years, commonly seen during weaning and post weaning period Marasmus: Patient with 60% of expected body weight without edema Kwashiorkor: Patient who weighs 60 - 80 % of expected body weight for that age with severe protein malnutrition with relative carbohydrate excess Marasmus: Clinical features Skin Dry, inelastic, thin, wrinkled, loose Follicular hyperkeratosis (adults) Hair Slow growth, lustreless Growth of lanugo hair occurs Nails : Fissured Facies : “Monkey facies”- wrinkled skin with loss of buccal fat pad Child is alert Kwashiorkor: Clinical features Skin “Flaky paint” or “crazy pavement” dermatoses, extensive peeling of skin with erosions “Enamel paint” dermatoses Sites: pressure sites Sharply demarcated hyperpigmented plaques with burnt out appearance and waxy feel (spares feet and dorsa of hands in contrast to pellagra) Clinical features Mucosae: Cheilitis with fissuring on lips Angular stomatitis, glossitis Nails: Soft and thin Hair : Sparse, thin, brittle, easy pluckable Dyschromotrichia : golden, blonde, rust (red boy) Flag sign Eyelashes : broomstick appearance Treatment High protein, high caloric diet Topical zinc paste, oral zinc supplements Correction of other associated deficiency Treatment of infection and infestation Thank you