Brandt syndrome (BS) is an autosomal recessive inborn error of metabolism that usually occurs in early childhood, particularly in children who have recently been weaned off breast milk, or a few weeks after birth in children who are not breast fed. Symptoms are primarily due to zinc deficiency, secondary to reduced absorption of zinc [1]. Acquired deficiency of this mineral is seen in adulthood, and the signs are the same as those of BS [2] [3] [4]. Zinc deficiency can be caused by various factors, like restricted diet, gastrointestinal pathologies such as celiac disease, alcoholism, excessive intake of phytates, burns and renal disease [5] [6].

The clinical triad for BS is dermatitis, diarrhea, and alopecia, although few patients present in this way [7]. Dermatitis characteristic of BS includes periorificial, around the mouth and eyes, and acral dermatitis, which affects the extremities. Skin lesions may be pustular, vesicular, erythematous and crusted. In addition, they have a clear demarcation and may resemble eczema or psoriasis. As the condition progresses, lesions may spread to other areas of the body such as the buttocks and thighs. Alopecia is noted in the hair as well as eyebrows and eyelashes. Other dermatological symptoms are dysmorphic nails and superimposed bacterial or fungal infection. The latter may make the correct diagnosis even more difficult to ascertain.

Involvement of mucosal surfaces may manifest in the form of blepharitis, conjunctivitis and inflamed gums and stomatitis [8]. Furthermore, patients may also show changes in mood, photophobia, increased susceptibility to infection, mental dullness, a reduced sense of taste and anorexia. In infants, additional symptoms comprise of failure to thrive, growth retardation and irritability.

Diagnosis of BS incorporates clinical assessment as well as laboratory studies. In a patient suspected to have BS because of their clinical symptoms, serum zinc levels, which may be depleted, can be calculated. A value less than 50 micro-grams per deciliter implies BS [5]. Measuring zinc in the hair and urine is also done, but this is not common [7]. Serum alkaline phosphatase levels may be low. If it is in the normal range, however, this does not rule out BS. Further, the administration of zinc and an improvement in symptoms is used to confirm the diagnosis.

Genetic testing for the mutation in the solute carrier family 39 member 4 (SLC39A4) gene are not routinely available.

Light and electron microscopy may be utilized in the analysis of biopsy samples. A breast feeding infant's mother can have her breast milk screened for low zinc, as an inadequate concentration in breast milk may be the cause of zinc deficiency in an infant [9].

Histological examination of lesions yields findings such as neutrophils in the epidermal layer as well as abnormal keratinization of the epidermis, pallor, tissue necrosis and hyperplasia. However, such details may also be noticed in other nutritional diseases.

The primary treatment for Acrodermatitis Enteropathica is zinc supplementation. This involves:

• Oral Zinc Supplements: Administered daily to maintain normal zinc levels in the body.
• Dietary Adjustments: Ensuring a diet rich in zinc, although supplements are usually necessary to meet the body's needs.

With proper treatment, symptoms typically improve rapidly, and long-term management is focused on maintaining adequate zinc levels.

With early diagnosis and appropriate treatment, the prognosis for individuals with Acrodermatitis Enteropathica is excellent. Zinc supplementation effectively manages symptoms and prevents complications. However, lifelong adherence to treatment is necessary, as discontinuation can lead to a recurrence of symptoms. Regular monitoring by healthcare professionals ensures optimal management and quality of life.

Acrodermatitis Enteropathica is caused by mutations in the SLC39A4 gene, which encodes a protein responsible for zinc transport in the intestines. These mutations impair the body's ability to absorb zinc from the diet, leading to the symptoms associated with the disorder. It is inherited in an autosomal recessive pattern, meaning both parents must carry a copy of the mutated gene for their child to be affected.

Acrodermatitis Enteropathica is a rare condition, with an estimated incidence of 1 in 500,000 births worldwide. It affects both males and females equally and is found in various ethnic groups. Due to its rarity, it may be underdiagnosed or misdiagnosed, highlighting the importance of awareness among healthcare providers.

The pathophysiology of Acrodermatitis Enteropathica involves impaired zinc absorption due to defective zinc transport proteins. Zinc is vital for numerous enzymatic reactions and cellular processes. Its deficiency affects the skin, gastrointestinal tract, and hair follicles, leading to the characteristic symptoms of the disorder. Zinc's role in immune function also explains the increased susceptibility to infections observed in affected individuals.

Currently, there is no known way to prevent Acrodermatitis Enteropathica, as it is a genetic disorder. However, early detection and treatment can prevent complications and improve outcomes. Genetic counseling may be beneficial for families with a history of the disorder to understand the risks and implications for future pregnancies.

Acrodermatitis Enteropathica is a rare genetic disorder characterized by impaired zinc absorption, leading to skin lesions, diarrhea, and hair loss. Early diagnosis and treatment with zinc supplements are crucial for managing symptoms and ensuring a good quality of life. Understanding the genetic basis and pathophysiology of the disorder aids in effective management and highlights the importance of awareness among healthcare providers.

For patients and families affected by Acrodermatitis Enteropathica, understanding the condition is key to effective management. It is a genetic disorder that affects zinc absorption, leading to symptoms like skin rashes, diarrhea, and hair loss. Treatment involves daily zinc supplements, which help manage symptoms and prevent complications. Regular follow-up with healthcare providers ensures optimal care and quality of life. Genetic counseling can provide valuable information for families regarding inheritance patterns and future pregnancies.

1. Weismann K, Hoe S, Knudsen L, Sørensen SS. 65Zinc absorption in patients suffering from acrodermatitis enteropathica and in normal adults assessed by whole-body counting technique. Br J Dermatol. 1979;101(5):573–579.
2. Wierdsma NJ, van Bokhorst-de van der Schueren MA, Berkenpas M, Mulder CJ, van Bodegraven AA. Vitamin and mineral deficiency are highly prevalent in newly diagnosed celiac disease patients. Nutrients. 2013;5(10):3975–3992.
3. Bae-Harboe YS, Solky A, Masterpol KS. A case of acquired zinc deficiency. Dermatol Online J. 2012;18(5):1.
4. Shahsavari D, Ahmed Z, Karikkineth A, Williams R, Zigel C. Zinc-deficiency acrodermatitis in a patient with chronic alcoholism and gastric bypass: a case report. J Community Hosp Intern Med Perspect. 2014;4(3).
5. Maverakis E, Fung MA, Lynch PJ, et al. Acrodermatitis enteropathica and an overview of zinc metabolism. J Am Acad Dermatol. 2007;56(1):116–124.
6. Chaudhry AA, Warthan MM, Praiser RJ, Hood AF. Acquired acrodermatitis enteropathica secondary to alcoholism. Cutis. 2008;82(1):60–62.
7. Van Wouwe JP. Clinical and laboratory diagnosis of acrodermatitis enteropathica. Eur J Pediatr. 1989;149(1):2–8.
8. Gutiérrez-González E, Alvarez-Pérez A, Loureiro M, Sánchez-Aguilar D, Toribio J. Acrodermatitis enteropathica in a breast-fed infant. Actas Dermosifiliogr. 2012;103(2):170–172.
9. Perafán-Riveros C, França LF, Alves AC, Sanches JA Jr. Acrodermatitis enteropathica: case report and review of the literature. Pediatr Dermatol. 2002;19(5):426–431.