Acrodermatitis Enterohepatica

Acrodermatitis Enterohepatica

Q. 1

Dose of zinc used in acrodermatitis enteropathica is?

 A

0.5mg/kg

 B

2mg/kg

 C

5mg/kg

 D

7mg/kg

Q. 1

Dose of zinc used in acrodermatitis enteropathica is?

 A

0.5mg/kg

 B

2mg/kg

 C

5mg/kg

 D

7mg/kg

Ans. B

Explanation:

 

Dose of zinc for acrodermatitis enteropathica is 2-3 mg/kg body weight


Q. 2

Which is false about acrodermatitis enteropathica –

 A >Triad of diarrhoea, dementia and dermatitis
 B >Low serum zinc levels.
 C >Symptoms improve with zinc supplementation
 D >Autosomal recessive
Q. 2

Which is false about acrodermatitis enteropathica –

 A >Triad of diarrhoea, dementia and dermatitis
 B >Low serum zinc levels.
 C >Symptoms improve with zinc supplementation
 D >Autosomal recessive
Ans. A

Explanation:

Triad of diarrhoea, dementia and dermatitis [Ref.- Harrison I7/e p. 449]

  • Acrodermatitis enteropathica causes diarrhoea and dermatitis but no dementia.
  • Acrodermatitis enteropathica is a rare autosomal recessive disorder caused by an inability to absorb sufficient Zinc from the diet.
  • It results in numerous clinical manifestations the variety of manifestations, of the syndrome may be due to the fact that zinc has a role in numerous metabolic pathways including those of copper, protein, essential .fatty acids and prostaglandins and zinc is incorporated into many zinc rnetalloenzymes.

Symptoms: ?

  • Initial signs and symptoms occur during the first few months of life often after weaning from breast to Cow’s milk. The main manifestations are: ?

Skin

  • Vesciobullous, eczematous, dry, scaly or psoriasiform skin lesions, symmetrically distributed in the perioral, acral and perineal areas and on the cheeks, knees and elbows.

Hair

  • Hair often has a peculiar reddish tint and alopecia of some degree is characteristic.

Occular manifestations

  • Photophobia
  • Conjunctivitis
  • Blepharitis
  • Corneal dysdrophy

Associated manifestations

  • Chronic diarrhoea
  • Stomatitis
  • Glossitis
  • Paronychia
  • Nail dystrophy
  • Growth retardation
  • Irritability
  • Delayed wound healing
  • Intercurrent bacterial infection and superinfection with candida albicans.

Diagnosis

  • Constellation of clinical findings.
  • Detection of low plasma Zinc concentration

Treatment : ?

  • Without tit the course is chronic and intermittent but often relentlessly progressive.
  • Oral therapy with Zinc compound is the tit of choice.
  • Zinc therapy rapidly abolishes the manifestation of the disease.

Q. 3

Which is false about acrodermatitis enteropathica?

 A

Triad of diarrhoes, dementia and dermatitis

 B

Low serum zinc levels

 C

Symptoms improve with zinc supplementation

 D

Autosomal recessive

Q. 3

Which is false about acrodermatitis enteropathica?

 A

Triad of diarrhoes, dementia and dermatitis

 B

Low serum zinc levels

 C

Symptoms improve with zinc supplementation

 D

Autosomal recessive

Ans. A

Explanation:

Triad of diarrhoea, dementia and dermatitis

  • Acrodermatitis enteropathica causes diarrhoea and dermatitis but no dementia.
  • Acrodermatitis enteropathica is a rare autosomal recessive disorder caused by an inability to absorb sufficient Zinc from the diet.
  • It results in numerous clinical manifestations the variety of manifestations, of the syndrome may be due to the fact that zinc has a role in numerous metabolic pathways including those of copper. protein, essential fatly acids and prostaglandins and zinc is incorporated into many zinc metalloenzymes.

Symptoms : 

  • Initial signs and symptoms occur during the first few months of life often after weaning from breast to Cow’s milk.

The main manifestations are 

Skin

  • Vesciobullous, eczematous, dry, scaly or psoriasiform skin lesions, symmetrically distributed in the

perioral, acral and perineal areas and on the cheeks, knees and elbows.

Hair

  • Hair often has a peculiar reddish tint and alopecia of some degree is characteristic.

Occular manifestations

  • Photophobia
  • Conjunctivitis
  • Blepharitis
  • Corneal dysdrophy

Associated manifestations

  • Chronic diarrhoea
  • Stomatitis
  • Glossitis
  • Paronychia
  • Nail dystrophy
  • Growth retardation
  • Irritability
  • Delayed wound healing
  • Intercurrent bacterial infection and superinfection with candida albicans. Diagnosis
  • Constellation of clinical findings.
  • Detection of low plasma Zinc concentration

Treatment : 

Without the course is chronic and intermittent but often relentlessly progressive.

Oral therapy with Zinc compound is the t/t of choice.

Zinc therapy rapidly abolishes the manifestation of the disease.

 [Ref: Harrison 171hIe p. 449]


Q. 4

Which of the following statement regarding Acrodermatitis enteropathica is true?

 A

Lifelong treatment required

 B

Autosomal dominant disorder

 C

Wound healing is not affected

 D

Zinc absorption is normal

Q. 4

Which of the following statement regarding Acrodermatitis enteropathica is true?

 A

Lifelong treatment required

 B

Autosomal dominant disorder

 C

Wound healing is not affected

 D

Zinc absorption is normal

Ans. A

Explanation:

Treatment of acrodermatitis enteropathica involve lifelong supplementation of Zinc.

Acrodermatitis enteropathica is an autosomal recessive disease characterised by defective absorption of zinc from the intestine.

It is mainly seen in breast fed babies whose mothers do not secrete zinc into the breast milk.

Zinc deficiency is associated with impaired granulation tissue formation which inturn result in impaired wound healing. 

It is characterized by impaired wound healing and as well as erythematous pustular dermatitis involving the extremities and body orifices.

It is diagnosed by the presence of abnormally low blood zinc levels (more than 100mcg/dl).


Q. 5

A 6 month old child presented with diarrhea for few days. He had dermatitic lesions on his hands and feet. Scalp hair was comparatively less. Deficiency of zinc was suspected. TRUE about acrodermatitis enteropathica is:

 A

Autosomal recessive

 B

Autosomal dominant

 C

Zinc treatment cures partially

 D

Treatment life long

Q. 5

A 6 month old child presented with diarrhea for few days. He had dermatitic lesions on his hands and feet. Scalp hair was comparatively less. Deficiency of zinc was suspected. TRUE about acrodermatitis enteropathica is:

 A

Autosomal recessive

 B

Autosomal dominant

 C

Zinc treatment cures partially

 D

Treatment life long

Ans. A

Explanation:

Acrodermatitis enteropathica (AE):

  • It is an autosomal recessive disorder of zinc absorption.
  • It presents in infancy, characterized by a triad of acral dermatitis (face, hands, feet, anogenital area), alopecia, and diarrhea.
  • These infants have a defect in an intestinal zinc transporter, the human ZIP4 protein encoded on the SLC39A4 gene. 
  • Mutations in this gene prevents appropriate enteral zinc absorption.
  • AE classically presents during infancy on weaning from breast milk to formula or cereal, which have lower zinc bioavailability than breast milk.
 
Ref: Jen M., Yan A.C. (2012). Chapter 130. Cutaneous Changes in Nutritional Disease. In L.A. Goldsmith, S.I. Katz, B.A. Gilchrest, A.S. Paller, D.J. Leffell, N.A. Dallas (Eds), Fitzpatrick’s Dermatology in General Medicine

Q. 6

Acrodermatitis entropathica is charecterized by all of the following, EXCEPT:

 A

Chronic diarrhoea

 B

Protein malabsorption

 C

Zinc defiency a possible etiology

 D

Denuded skin around mucocutaneous junction

Q. 6

Acrodermatitis entropathica is charecterized by all of the following, EXCEPT:

 A

Chronic diarrhoea

 B

Protein malabsorption

 C

Zinc defiency a possible etiology

 D

Denuded skin around mucocutaneous junction

Ans. B

Explanation:

Acrodermatitis enteropathica is a rare autosomal recessive disease caused by an inability to absorb sufficient zinc from diet.

Genetic is in the intestinal Zinc specific transporter gene SLC39A4 located on chromosome 8.

Zinc deficiency manifests with growth retardation, alopecia, dermatitis, chronic diarrhea, rashes around mucocutaneous junction and on the extremities.

Ref: Inborn Metabolic Diseases: Diagnosis and Treatment, edited by John ernandes, Page 472


Q. 7

A child with alopecia, hyperpigmentation psoriatic dermatitis in genitals & mouth and hypogonadism is likely to be suffering from –

 A

Cu defeciency

 B

Iron deficiency

 C

Zn deficiency

 D

Mg deficiency

Q. 7

A child with alopecia, hyperpigmentation psoriatic dermatitis in genitals & mouth and hypogonadism is likely to be suffering from –

 A

Cu defeciency

 B

Iron deficiency

 C

Zn deficiency

 D

Mg deficiency

Ans. C

Explanation:

Ans. is ‘c’ i.e., Zinc deficiency

Features of Zinc deficiency include  :

1. Dwarfism (growth retardation)               5. Iron deficiency anemia                        9. Hyperpigmentation

2.   Diarrhea                                         6. Acrodermatitis enteropathica               10. Hypogonadism

3. Dermatitis                                        7. Decreased immunocompetance            11. Perioral rash

4. Hepatosplenomegaly                           8. Poor wound healing

o Loss of hair and taste alterations may also be seen.

The patient in question presents with hyperpigmentation, hypogonadism, dermatitis and alopecia, all of which may be seen with Zinc deficiency.


Q. 8

All are true about Achrodermatitis enteropathica except: 

 A

I,Zn level (low serum zinc level)

 B

Reverse with Zn supplement

 C

Triad of acral dermatitis, dementia & diarrhea

 D

AR

Q. 8

All are true about Achrodermatitis enteropathica except: 

 A

I,Zn level (low serum zinc level)

 B

Reverse with Zn supplement

 C

Triad of acral dermatitis, dementia & diarrhea

 D

AR

Ans. C

Explanation:

C i.e. Triad of acral dermatitis , dementia & diarrhea 


Q. 9

Dermatitis and alopecia are due to deficiency of:

 A

Zinc

 B

Molybodenum

 C

Magnesium

 D

Calcium

Q. 9

Dermatitis and alopecia are due to deficiency of:

 A

Zinc

 B

Molybodenum

 C

Magnesium

 D

Calcium

Ans. A

Explanation:

A i.e. Zinc


Q. 10

Acrodermatitis entero pathica is d/t deficiency of:

 A

Zn

 B

Se

 C

Cu

 D

Cr

Q. 10

Acrodermatitis entero pathica is d/t deficiency of:

 A

Zn

 B

Se

 C

Cu

 D

Cr

Ans. A

Explanation:

A. i.e. Zn

Autosomal recessiveQ disorder acrodermatitis enteropathica d/t deficiency of zinc0 is characterized by diarrhea, depression, eczematous dermatitis, alopecia and lethargy. (Mn- DEAL). Zn supplementation rapidly improves conditionQ.


Q. 11

Acrodermatitis hemorrhagica is due to deficiency of

 A

Zinc

 B

Manganese

 C

Copper

 D

Selenium

Q. 11

Acrodermatitis hemorrhagica is due to deficiency of

 A

Zinc

 B

Manganese

 C

Copper

 D

Selenium

Ans. A

Explanation:

Ans. is ‘a’ i.e., Zinc

Symptoms of Zinc Deficiency

Mild deficiency                   Severe dificiency

Growth retardation                Dwarfism

                                          Cardiomyopathy

                                          Hypogonadism

                                          Infertility

                                          Loss of taste

                                          Poor wound healing

                                          Deformed bones

                                          Diarrhoea

                                          Alopecia

                                          Night blindness

                                          Skin striae

                                          Nail changes

  • Acrodermatitis enteropathica is an inherited autosomal recessive disorder with imapired intestinal disorder and transport of Zinc.
  • Patient suffers with pustular and bullous dermatitis, alopecia, growth retardation diarrhoes, secondary infection, lethargy, irritability and depression. Oral Zinc supplementation leads to remission.

Zinc

  • 2nd most abundant trace element in the body.
  • Most common catalytic metal ion in the cell cytoplasm.
  • Component of more than 100 enzymes like DNA polymerase, RNA polymerase, transfer RNA synthetase.
  • It plays role in all stages of insulin metabolism.


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