Acrodermatitis Enteropathica

Acrodermatitis Enteropathica is a metabolic disorder and is autonomic recessive. This disease affects the zince uptake which is characterized by periorificial or around the natural orifice and in the limbs or acral dermatitis, diarrhea and loss of hair or alopecia. There is also a similar feature presented in the acquired zinc deficiency disorder. It is as well related to zinc insufficiency due to congenital cause. This condition is a rare one which is inherited from a member of a family.

Other Names of Acrodermatitis Enteropathica

1. Danbolt-Cross Syndrome
2. Brandt Syndrome
3. Congenital Zinc Deficiency

You may wonder who are the most affected of this kind of an uncommon condition. This primarily affects young children and infants. It appears as red or brown rashes on various parts of the body such as face, feet, hands and buttocks. Most of the cases are acute, it means that the rashes tend to appear suddenly that persist in just a couple of days. However, this condition might as well a long-term or an occasional problem.

The doctors often associate this problem with viral and bacterial pathogens. The growth of acrodermatitis is found to be caused by the said pathogens. On the other hands, the treatments for this kind of condition also vary. This is being treated depending on the underlying cause. However, most often, this is being addressed by oral and topical medications and allows infected children to recover with in few weeks from the start of infection.

One of the vital components of the diet is zinc. This is also found on the breast milk and in fact, this kind of zinc is very absorbable compared to those found in other milk formulas. Zinc is also found in shellfish, meat and wheat germ. On the other hand, that zinc found on foods of plant origin is having low levels of this vitamin. Zinc is very helpful in assisting metalloenzymes in the body. This metalloenzymes are responsible in various important cellular processes like in the production of anti-inflammatory agents. Examples of which are antioxidants and cytokines.

Acrodermatitis Enteropathica Signs and Symptoms

1. The features or signs of Acrodermatitis Enteropathica usually start appearing in the initial months of life as the infant starts to stop being breastfed.
2. There are plaques of dry, erythematous patches and scaly skin. The lesions are as well appearing and evolving eczematous, pustules and crusted bullas or vesicles.
3. These lesions are also found around the anus and mouth as well as on the feet, scalp and hands.
4. Suppurative inflammation on the folds of nails would also likely to occur surrounding the nail plates. It is commonly known as “paronychia”.
5. Loss of hair scalp or Alopecia can also occur as well as the hairs on the eyelashes and eyebrows.
6. These skins lesions are just secondary infections caused by the Staphylococcus aureus bacteria or fungi such as Candida Albicans.
7. These kin lesions are most often accompanied by diarrhea.

Picture : Acrodermatitis on foot

Genetics

One thing that is responsible in this condition is the mutation of the gene SLC39A4 on the 8th chromosome. The gene is the one responsible in encoding the transmembrane protein which is serving a zinc uptake protein. This disease often manifests starting at infant when it is weaned from breastfeeding. This has lead to the scientists to suspect that breast milk contain beneficial substance that aids in the zinc absorption and in return prevent the disease from manifesting.

Treatment for Acrodermatitis Enteropathica

Acrodermatitis Enteropathica when left untreated might be fatal and could kill an individual with in few years only. It has been finalized that this kind of disease as no cure, it is just that the treatment for this kind of condition is a lifelong zinc dietary supplement with a range of 1 to 2 mg/kg of the patient’s bodyweight daily.

Pathophysiology

An essential trace nutrient that is required for the proper functioning of more than a hundred enzymes is zinc. It also plays a crucial role in the metabolism of the nucleic acid. The difference between acquired zinc deficiency disorder and Acrodermatitis Enteropathica is real difficult since they are both having the same clinical presentations.

The acquired zinc deficiency disorder is mainly the result if poor nutrition or low nutritional intake, excessive loss of zinc, malabsorption or a combination of the mentioned factors.

On the other hand, Acrodermatitis Enteropathica can only be diagnosed properly after zinc supplementation attempts failed. Most importantly, the transient acquired zinc deficiency can occur in premature infants following to their increased physiological demands for zinc and minimal body stores. In addition, deficiency in zinc can present in full-term breastfeeding infants as an effect of stumpy levels of zinc serum due to a defective zinc secretion on the mammary gland.

Acrodermatitis Enteropathica Epidemiology

In the United States, the Acrodermatitis Enteropathica frequency is still unknown. On the other hand, for is international estimation, there are only 1 person infected by this disease for every 500,000 people. This is true with Denmark.

Without the appropriate and right supplementation of zinc, Acrodermatitis Enteropathica can always be lethal especially during the early years in life. Infants that are left untreated might exhibit dermatitis, severe growth retardation, secondary bacterial and fungal infection, alopecia and changes in the behavior. Consequently, all symptoms can be reversible when applied with proper therapy.

Modifications

• Acrodermatitis Enteropathica disease has no racial and sex predilection.
• It usually appears during infancy.
• It can occur on children who are on breastfeeding where there are low levels of zinc on the breast milk.

The Other Symptoms of Acrodermatitis Enteropathica

• Aside from the major clinical features mentioned above, this condition has also other features like conjunctivitis or sore eyes
• Light sensitivity
• Loss of appetite or anorexia
• Mild to severe diarrhea
• Irritability in which the baby whine incessantly and cry
• Depressed mood and growth  failure

In suspecting for a zinc deficiency, there are helpful investigations that can help in leading the real condition.

1. Anemia is possible if blood count will be analyzed.
2. In order to come up with the characteristic features, skin biopsy might be needed.
3. There are as well reduced urinary zinc secretions.
4. If the normal level of zinc is not met with in the diagnosis ( plasma/serum zinc levels: normal range is 10.7 – 23.0 µmol/L)

References:

emedicine.medscape.com/article/1102575-overview