SEMINAR ON NUTRITIONAL DISORDERS OF SKINSEMINAR ON NUTRITIONAL DISORDERS OF SKIN

Chairperson : Professor (Dr.) C.N. Sarker Head of the department

Department of Medicine, MMC.

Speakers : 1. Dr. Md. Nazrul Islam, MCPS,DDV,FCPS (Part-II)

2. Dr. Mohammed Saiful Islam Bhuiyan, MD (Part-II), FCPS (Part-II) Medical Officers,

3. Dr. Mohammad Assaduzzaman MCPS, FCPS (Part-II) Assistant Registrar Department of Dermatology, MMCH.

Date & Time : 15th May, 2005 at 1.30 pm.

Organized by : Department of Dermatology, MMCH. &

SK+F

INTRODUCTIONINTRODUCTION

A large number of disorders in relation to

nutrition are being found in our profession.

If we want to know about them we’ll have to

go through some basic terms.

FOODFOOD

Food is any substance which an individual Food is any substance which an individual

takes, digests and assimilates to derive takes, digests and assimilates to derive

nutritive requirements for maintaining nutritive requirements for maintaining

growth and physical well – being.growth and physical well – being.

NUTRITION

Nutrition is a dynamic process

concerned with ingestion , digestion,

absorption and assimilation of food

for nourishing the body.

SOME SIGNS OF GOOD NUTRITIONSOME SIGNS OF GOOD NUTRITION

Smooth shiny skin.Glossy hair. Well developed muscles, bones and

teeth.Strong built and energetic to look at.

NUTRIENTS

Nutrients are the constituents of food necessary to sustain the normal function of the body.1. Macronutrients

CHO, protein, fat, Ca, Na, K, Mg, Cl & PO4.

2. Micronutrients

Vitamins, trace elements.

ESSENTIAL NUTRIENTSESSENTIAL NUTRIENTS

Essential nutrients are those that

either cannot be synthesized in

the body or cannot be synthesized

in adequate amount to meet the

needs of the body.

THE ESSENTIAL NUTRIENTS

1. Certain amino acids.

2. Certain fatty acids.

3. Vitamins and

4. Minerals.

VITAMINSVITAMINSVitamins are organic nutrients that Vitamins are organic nutrients that are required in small quantities for a are required in small quantities for a variety of biochemical functions and variety of biochemical functions and which, generally, can not be which, generally, can not be synthesized by the body and must synthesized by the body and must therefore be supplied bytherefore be supplied bythe diet.the diet.

TYPES OF VITAMINS

1. Fat soluble vitamins.- Vitamin A, D, E & K.

2. Water soluble vitamins.- Vitamin B –Complex and C.

MINERALS

Minerals are inorganic elements or

substances required by the organism in very

small amounts for maintenance of vital

processes essential for life.

TYPES OF MINERALSTYPES OF MINERALS1. 1. Principal elements/macromineralsPrincipal elements/macrominerals K, Ca, Mg, Na, P, S and Cl.K, Ca, Mg, Na, P, S and Cl.2. 2. Trace elements/ micromineralsTrace elements/ microminerals

Iron , Copper, Fluoride, Iodine, Iron , Copper, Fluoride, Iodine, Cobalt, Cobalt, Zinc, Molybdenum,Zinc, Molybdenum,

Selenium, Selenium, Silicon,Silicon, Nickel, Nickel, Tin, Cromium, Vanadium.Tin, Cromium, Vanadium.

NUTRITIONAL DISORDERNUTRITIONAL DISORDERCLASSIFICATIONCLASSIFICATION

1.1. Undernutrition. Undernutrition. 2.2. Malnutrition.Malnutrition.3.3. Nutrient excess.Nutrient excess.4.4. Obesity.Obesity.5.5. Effect of toxicants in foods.Effect of toxicants in foods.

DISORDERS DUE TO VITAMIN DISORDERS DUE TO VITAMIN DEFICIENCYDEFICIENCY

Vitamin AVitamin A Phrynoderma, xerophthalmia, Phrynoderma, xerophthalmia, night blindness,keratomalacia.night blindness,keratomalacia.

Vitamin DVitamin D Rickets, osteomalaciaRickets, osteomalacia

Vitamin EVitamin E Haemolytic anaemia, ataxiaHaemolytic anaemia, ataxia

Vitamin KVitamin K Purpura, haemorrhage, Purpura, haemorrhage, ecchymosis.ecchymosis.

DISORDERS DUE TO VITAMIN DISORDERS DUE TO VITAMIN DEFICIENCY (Contd.)DEFICIENCY (Contd.)

Thiamin (Vitamin B1)Thiamin (Vitamin B1) Beri-beriBeri-beri

Riboflavin Riboflavin (VitaminB(VitaminB22))

Oro-ocular-genital Oro-ocular-genital syndrome (glossitis, syndrome (glossitis, stomatitis etc.)stomatitis etc.)

Niacin (Vitamin BNiacin (Vitamin B33)) Pellagra.Pellagra.

Vitamin BVitamin B6 6

(Pyridoxine)(Pyridoxine)PolyneuropathyPolyneuropathy

BiotinBiotin Dermatitis, alopecia,Dermatitis, alopecia,paraesthesiae.paraesthesiae.

DISORDERS DUE TO VITAMIN DEFICIENCY (Contd.)

Folate Anaemia

Vitamin B12 (Cobalamin)

Glossitis, hyperpigmentation, canitis.

Vitamin-C (Ascorbic acid)

Scurvy

DISORDERS DUE TO DISORDERS DUE TO MINERAL DEFICIENCYMINERAL DEFICIENCY

IronIron Anaemia, glossitis, Anaemia, glossitis, cheilosis, cheilosis, koilonychia.koilonychia.

ZincZinc Acrodermatitis Acrodermatitis enteropathicaenteropathica

ESSENTIAL AMINO ACID AND ESSENTIAL AMINO ACID AND ESSENTIAL FATTY ACID ESSENTIAL FATTY ACID DEFICIENT DISORDERSDEFICIENT DISORDERS

MarasmusMarasmus Sprue Sprue

Kwarshiorkor Kwarshiorkor CarotenemiaCarotenemia

Pellagra Pellagra LycopenemiaLycopenemia

DISORDERS DUE TO NUTRIENTS DISORDERS DUE TO NUTRIENTS EXCESSEXCESSVitamin A Liver damage, bone

damage, teratogenesisVitamin D Hypercalcaemia

Vitamin B6

(pyridoxine)Subepidermal vesicular dermatitis, sensoryperipheral neuropathy

Iron Haemosiderosis

KERATOMALACIA

ANGULAR STOMATITIS

PELLAGRA

SCURVY

MARASMUS

KWARSHIORKOR

PHRYNODERMA

ACRODERMATITIS ENTEROPATHICA

OBESITY

An alarming issue. A nutritional & metabolic disorder. Results from excessive intake of food

& insufficient exercise. It is said “rich people of poor countries

& poor people of rich countries” are the sufferers.

OBESITY

“EXCESS OF ANYTHING IS BAD”

ACRODERMATITIS ENTEROPATHICA

ACRODERMATITIS ACRODERMATITIS ENTEROPATHICAENTEROPATHICA

Acrodermatitis enteropathica (AE) is a rare inherited disorder transmitted as an autosomal recessive trait, caused by defective intestinal absorption of Zn, characterized by a triad of acral dermatitis, alopecia and diarrhea.

EpidemiologyEpidemiology:: No geographical, racial or genderNo geographical, racial or gender

predilection.predilection.

Etiology:Etiology:

Genetic – autosomal recessive, defect inGenetic – autosomal recessive, defect in

intestinal zinc absorption.intestinal zinc absorption.

CONDITIONS MAY CAUSE CONDITIONS MAY CAUSE ZINC DEFICIENCYZINC DEFICIENCY

Reduced intake Reduced intake Anorexia nervosa.Anorexia nervosa.Bulimia.Bulimia.Faddish weight reduction.Faddish weight reduction.Prolong total parenteral nutrition.Prolong total parenteral nutrition.High dietary phytate.High dietary phytate.

FF

CONDITIONS MAY CAUSE CONDITIONS MAY CAUSE ZINC DEFICIENCY (Contd.)ZINC DEFICIENCY (Contd.)

Reduced absorption.Reduced absorption.Mucosal disease.Mucosal disease.

Malabsorption syndromes.Malabsorption syndromes.

Pancreatic disorders.Pancreatic disorders.

CONDITIONS MAY CONDITIONS MAY CAUSE ZINC CAUSE ZINC

DEFICIENCY (Contd.)DEFICIENCY (Contd.)Increased lossIncreased lossMalabsorption syndrome.Malabsorption syndrome.Blind-loop syndrome.Blind-loop syndrome.Renal tubular disease.Renal tubular disease.Nephrotic syndrome.Nephrotic syndrome.Dialysis. Dialysis. Diabetes mellitus.Diabetes mellitus.

CONDITIONS MAY CAUSE ZINC CONDITIONS MAY CAUSE ZINC DEFICIENCY (Contd.)DEFICIENCY (Contd.)

Increased Catabolism Increased Catabolism Malignancy.Malignancy.Burns.Burns.Postsurgical procedure.Postsurgical procedure.Antimetabolite drug therapy.Antimetabolite drug therapy. Increased DemandIncreased Demand Pregnancy.Pregnancy.Lactation.Lactation.

SOURCE OF ZINC

Meat.

Liver.

Egg.

Seafood.

BIOCHEMISTRYOF ZINC BIOCHEMISTRYOF ZINC METABOLISM:METABOLISM:

Adult body contains 2-3 gms ofAdult body contains 2-3 gms of zinc, zinc, which iswhich is about half the ironabout half the iron content, 10-20 times more than content, 10-20 times more than other essential trace elements. other essential trace elements.

BIOCHEMISTRYOF ZINC BIOCHEMISTRYOF ZINC METABOLISM(Contd.)METABOLISM(Contd.)

Recommend dietary allowance: Recommend dietary allowance: AdultAdult - 15 mg/day (elemental). - 15 mg/day (elemental).Infants Infants - 5mg/day. - 5mg/day.ChildrenChildren - 10mg/day.- 10mg/day.Lactating mother,Lactating mother, 1-6 months1-6 months- 19 mg/day- 19 mg/day > 6 months> 6 months - 16 mg/day- 16 mg/day

ZINC METABOLISM (CONTD.)

Normally about 30% of daily intake is absorbed. All body tissue contain zinc. Richest Stores: Muscle, bone and prostate. Incorporation: > 300 metalloenzymes. Free radical scavenger: protect against oxidative damage.

TYPES OF ZINC DEFICIENCYTYPES OF ZINC DEFICIENCY

1.1. Hereditary type.Hereditary type.2.2. Non-hereditary type.Non-hereditary type.

Low grade, marginal, nonhereditaryLow grade, marginal, nonhereditaryzinc deficiency is far more commonzinc deficiency is far more commonthan the hereditary form.than the hereditary form.

PATHOGENESIS:

The defect in AE is somewhere in the

early stages of zinc absorption.

Zn absorption is partially reduced in AE.

PATHOGENESIS (Contd.)

Bioavailability of zinc is more in human

milk than bovine milk.

In AE, genetic defect in different zinc

transporter (Zn T-1 to T-4) as well as

intestinal zinc transporter.

PATHOGENESIS (Contd.)PATHOGENESIS (Contd.)

Biologically active Zinc is highly Biologically active Zinc is highly charged species that cannot cross cell charged species that cannot cross cell membrane by passive diffusion but the membrane by passive diffusion but the process can greatly enhanced when process can greatly enhanced when zinc is complexed with zinc binding zinc is complexed with zinc binding ligands (ZBL).ligands (ZBL).

PATHOGENESIS (Contd.)PATHOGENESIS (Contd.)Zinc in human milk is preferentially Zinc in human milk is preferentially

bound to lower molecular-weight bound to lower molecular-weight ligands (ligands (~~10,000) than bovine milk.10,000) than bovine milk.

Total protein of human milk Total protein of human milk (5.3mg/ml) compared with bovine (5.3mg/ml) compared with bovine milk (29mg/ml) influence the bio-milk (29mg/ml) influence the bio-availability of zinc in an unknown availability of zinc in an unknown mechanism.mechanism.

PATHOGENESIS (Contd.)PATHOGENESIS (Contd.)

Metallothioneines regulates the transport of zinc Metallothioneines regulates the transport of zinc

into the circulation and then to the liver and into the circulation and then to the liver and

kidneys. kidneys.

Zinc is component of some peptidases, important Zinc is component of some peptidases, important

for digestion of proteins in GIT. for digestion of proteins in GIT.

PATHOGENESIS (Contd.)

Keratinosome contain several zinc-

dependent enzyme systems, the

metabolism of which may be affected in

zinc deficiency.

Possible role of biotin in AE, particularly in

premature infants with Zn deficiency.

ZINC AND GENETICSZINC AND GENETICS

Genetic locus for acrodermatitis entropathica

on chromosome 8q24.3.

Nuclei are rich in Zinc, critically involved in

maintaining genitic stability, gene

expression, and cell proliferation,

defferentiation and death.

ZINC AND IMMUNITYZINC AND IMMUNITY Reduced immune function specially cellular.Reduced immune function specially cellular.

In Acrodermatitis enteropathica thymic In Acrodermatitis enteropathica thymic

atrophy causing depressed thymocyte and atrophy causing depressed thymocyte and

cellular immune function, particularly T-cell. cellular immune function, particularly T-cell.

Neutrophil, peripheral blood monocytes, Neutrophil, peripheral blood monocytes,

tissue macrophages and mast cells require tissue macrophages and mast cells require

optimal concentration of zinc for normal optimal concentration of zinc for normal

function. function.

CLINICAL MANIFESTATIONCLINICAL MANIFESTATIONAge of onset: Age of onset:

Infants with bovine milk- 4-10 weeks,Infants with bovine milk- 4-10 weeks, Breast fed baby – after weaning.Breast fed baby – after weaning.

Prominent feature:Prominent feature:- Dermatitis (Acral and periorificial- Dermatitis (Acral and periorificial-

pathognomic).pathognomic).- Diarrhea.Diarrhea.- Alopecia (Generalized).Alopecia (Generalized).- Crying baby.Crying baby.

OTHER MANIFESTATION:OTHER MANIFESTATION:

Perleche- common early sign Perleche- common early sign and a sign heralding relapse.and a sign heralding relapse.

Superficial oral aphthous–like Superficial oral aphthous–like lesions.lesions.

Photophobea.Photophobea.

CLINICAL MANIFESTATION CLINICAL MANIFESTATION (CONTD.)(CONTD.)

Paronychia and brightly erythematous

dermatitis of the palmer and finger

creases.

Nail dystrophy.

CLINICAL MANIFESTATION CLINICAL MANIFESTATION (CONTD.)(CONTD.)

Secondary infection with bacteria Secondary infection with bacteria and Candida albicans. and Candida albicans. Delayed wound healing Delayed wound healing Patients are irritable and Patients are irritable and emotionally labile.emotionally labile.

CLINICAL MANIFESTATION CLINICAL MANIFESTATION (CONTD.)(CONTD.)

Anorexia,Anorexia, hypogeusia, hypogeusia, hyposmia and anemia hyposmia and anemia

LONG TERM SEQULAE OF ZN LONG TERM SEQULAE OF ZN DEFICIENCYDEFICIENCY

Growth reterdation and dwarfism.Growth reterdation and dwarfism.

Delayed puberty.Delayed puberty.

Hypogonadism in male adolescent.Hypogonadism in male adolescent.

Continuing dermatitis.Continuing dermatitis.

LONG TERM SEQULAE (CONTD.)LONG TERM SEQULAE (CONTD.)

Frequent infection.Frequent infection.

Lower fertility.Lower fertility.

Lower fertility and congenital Lower fertility and congenital

malformation occurs in children of malformation occurs in children of

mothers with AE.mothers with AE.

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

Candidiasis.Diaper dermatitis.Pellagra.Necrolytic migratory erythema.

HISTOLOGY

Vacuolation at upper str. malphigi.

Vacules become confluent forming a subcorneal bulla.

Total epidermal necrosis with subepidermal blister formation.

Neutrophils typically present.

LABORATORY DIAGNOSISLABORATORY DIAGNOSIS Serum Zinc levels (80-120gm/dl).

-is reduced.

-most accurate, earliest, commonly used.

Erythrocyte and leukocyte zinc level

-is reduced

-quite sensitive to early minor changes in

total body Zn, but expensive.

LABORATORY LABORATORY DIAGNOSIS(Contd.)DIAGNOSIS(Contd.)

Urine level (200-500 mg/24 hrs.).

- is reduced.

Serum alkaline phosphatase

- is reduced.

- moderately sensitive.

Hair Zn level – long term Zn status.

TREATMENTTREATMENT

Dietary or intravenous supplimentation Dietary or intravenous supplimentation with zinc salt – 3mg/kg/day life long.with zinc salt – 3mg/kg/day life long.

ZINC TOXICITYZINC TOXICITY

Acute : Acute : Gastric irritation.Gastric irritation.Nausea.Nausea.Vomiting.Vomiting.Gastric heamorrhage.Gastric heamorrhage.

Chronic: Chronic: Reduced growth rates.Reduced growth rates.Reduced rate of reproduction.Reduced rate of reproduction.Anemia.Anemia.Hypo cupremia.Hypo cupremia.

PHRYNODERMAPHRYNODERMA

Other names: Other names: Hypovitaminosis –A. Hypovitaminosis –A. Toad skin,Toad skin,

DEFINITION DEFINITION

Phrynoderma is a condition characterized by–Excessive dryness , –Wrinkling –Scaling of skin –Follicular hyperkeratosis.

DEFINITION (CONTD.)DEFINITION (CONTD.)•Due to deficiency of Vitamin- A.Due to deficiency of Vitamin- A.•May be associated with deficiency of May be associated with deficiency of

- Vitamin – B-Complex.- Vitamin – B-Complex.- Vitamin – C.- Vitamin – C.- Vitamin – E.- Vitamin – E.- Essential Fatty acids.- Essential Fatty acids.- Calories. - Calories.

INTRODUCTION

• Other names of vitamin A are retinol , anti infective vitamin

• Vitamin A is found in 2 forms animal food as retinol & vegetables as beta carotene

• Daily requirement of retinol -750 microgram in adult.

INTRODUCTION INTRODUCTION (CONTD.)(CONTD.)

1 microgram of retinol =6 microgram 1 microgram of retinol =6 microgram of beta caroteneof beta carotene

Vitamin A is measured in Vitamin A is measured in international unit where 1 IU=0.3 international unit where 1 IU=0.3 microgram retinolmicrogram retinol

About 90% of the body’s vitamin A About 90% of the body’s vitamin A reserve is found in the perisinusoidal reserve is found in the perisinusoidal stellate (Ito) cells in the liver. stellate (Ito) cells in the liver.

INTRODUCTION INTRODUCTION (CONTD.)(CONTD.)

About 90% of the body’s vitamin A reserve is found in the perisinusoidal stellate (Ito) cells in the liver.

Retinol is the transport & also the storage form of vitamin A.

INCIDENCE & PREVALENCE INCIDENCE & PREVALENCE

Vitamin A deficiency is common in Vitamin A deficiency is common in children in developing countries.children in developing countries.Specially in Asia & Africa & Specially in Asia & Africa & In such region most common cause In such region most common cause of blindness.of blindness.

INCIDENCE & PREVALENCE INCIDENCE & PREVALENCE

It is rare in developed countries & It is rare in developed countries & most commonly associated with most commonly associated with Fat malabsorption syndrome , Fat malabsorption syndrome , Mineral oil laxative abuse &Mineral oil laxative abuse & Mineral oil laxative abuse & Mineral oil laxative abuse &

ASSOCIATED DISORDERS OF VITAMIN A DEFICIENCY:• Diseases of fat malabsorption

syndrome•Crohns disease•Celiac disease •Cystic fibrosis •Cholestatic liver disease

ASSOCIATED DISORDERS OF ASSOCIATED DISORDERS OF VITAMIN A DEFICIENCY VITAMIN A DEFICIENCY

(CONTD.)(CONTD.)Inflammatory disorders –Inflammatory disorders –

Diarrhea, Diarrhea, PneumoniaPneumonia

CAUSES OF VITAMIN A CAUSES OF VITAMIN A DEFICIENCYDEFICIENCY

►Principal cause is inadequate Principal cause is inadequate diet(chiefly in 3diet(chiefly in 3rdrd world countries) world countries)

►Fat malabsorptionFat malabsorption►Liver damageLiver damage

PATHOLOGY & PATHOLOGY & PATHOGENESISPATHOGENESIS

• Vitamin A deficiency involves-•Visual system• Immune system•Skin •GIT•Respiratory system•Urinary system

SITES OF INVOLVEMENT SITES OF INVOLVEMENT IN SKININ SKIN

• 80%cases limited to – Arms – Arround elbows & knees

SITES OF INVOLVEMENT IN SITES OF INVOLVEMENT IN SKINSKIN

20% cases20% casesanterolateral aspect of thigh , anterolateral aspect of thigh , posterolateral aspect of posterolateral aspect of

superior forearm , superior forearm , the extensor surface of upper the extensor surface of upper

& lower extremities , & lower extremities , the shoulders , the shoulders , back back abdomen & abdomen & buttockbuttock

CLINICAL FEATURES IN SKINCLINICAL FEATURES IN SKIN

Skin becomes

–Dry

–Wrinkled,

–Covered with fine scale,

CLINICAL FEATURES IN CLINICAL FEATURES IN SKIN (CONTD.)SKIN (CONTD.)

Morphology of the lesion is variable Morphology of the lesion is variable may range from filiform papule to may range from filiform papule to small conical papule to large papule small conical papule to large papule with large horny centre ,with large horny centre ,

CLINICAL FEATURES IN SKIN (CONTD.)

Color may similar to surrounding skin or

may be slightly hyper pigmented

Back ground skin in affected area is

wrinkled

Hands & feet are not involved.

INVOLVEMENT OF VISUAL SYSTEM

In vitamin A deficiency eye findings are prominent & often pathognomonic.

• Delayed dark adaptation & night blindness is the earliest symptoms of vitamin A deficiency

INVOLVEMENT OF VISUAL INVOLVEMENT OF VISUAL SYSTEM (CONTD.)SYSTEM (CONTD.)

Dryness of the conjunctiva Dryness of the conjunctiva

(xeropthalmia) & development Bitot spot (xeropthalmia) & development Bitot spot

( small white patches on conjunctiva) ( small white patches on conjunctiva)

are early signs.are early signs.

INVOLVEMENT OF VISUALSYSTEM (CONTD.)

Keratomalacia ulceration & necrosis of the cornea, perforating endopthalmitis & blind ness are late manifestation .

HISTOPATHOLOGICAL HISTOPATHOLOGICAL FINDINGSFINDINGS

In adults :In adults :Follicular Hyperkeratosis Follicular Hyperkeratosis Follicular plugging in the upper Follicular plugging in the upper

portion of the hair follicle, portion of the hair follicle, Severe atrophy of the Severe atrophy of the

sebaceous gland.sebaceous gland.

HISTOPATHOLOGICAL FINDINGS(CONTD.)

Squamous metaplasia of the secretory cells of the eccrine sweat gland

Dermis is otherwise normal except for perifollicular infiltrate.

HISTOPATHOLOGICAL HISTOPATHOLOGICAL FINDINGS(CONTD.)FINDINGS(CONTD.)

• In infants & young adults –

(before pilosebaceous gland mature fully)–Simple xerosis or xeroderma is usually

characteristic feature

DIAGNOSIS DIAGNOSIS

Diagnosis of vitamin A Diagnosis of vitamin A deficiency is usually based on deficiency is usually based on The typical clinical findings & The typical clinical findings & Also aided by determining of serum Also aided by determining of serum vitamin A level (normal 30-65 mg/dl).vitamin A level (normal 30-65 mg/dl).

DIFFERENTIAL DIAGNOSIS OF PHRYNODERMA:

Keratosis pilarisDarier’s diseasePityriasis rubra pilarisAcne vulgaris Lichen planopilaris.

MANAGEMENTMANAGEMENT General management – General management –

improvement of diet. improvement of diet. Antibiotic if there is infection.Antibiotic if there is infection.

Special –Special –Treatment with vitamin A in Treatment with vitamin A in

deficient cases. deficient cases. Treatment with other specific Treatment with other specific

vitamins according to need. vitamins according to need.

TREATMENT (CONTD.) Phrynoderma -

– Local application of 10-40 % Urea cream

&

– 50,000 units of Vitamin A twice a day

orally can make the lesion disappear in 2-

3 months.

PREVENTION PREVENTION

• Oral dose of 60 mg retinol (200000 IU)

as palmitate given to preschool children

• Oral administration 4- 6 months

interval is sufficient to prevent serious

consequence of vitamin a deficiency.