The patient was a 21-month old Caucasian boy with normal birth weight who had a history of acral lesions and periorificial dermatitis since he was 3 months old. He was breastfed until he was two and a half months old. He has a history of upper respiratory tract infections and otitis media which was treated in a private clinic. His parents were blood-related and his older brother, who had the same symptoms, had died at the age of 14 months. His preliminary skin examination showed plaque like scaly skin, erythematous, dry skin, and skin lesions on areas around the eyes, mouth, and diaper area. Additionally, there were reports of hair loss (alopecia), soft tissue infections around the nails (paronychia), and cracks and infection in the inguinal region. Physical examinations stated that the infant irritable and photophobic. His measured height and weight were 68 cm and 7.8 kg, respectively.

The infant patient was admitted to another hospital because of his chronic intermittent diarrhea, where his skin lesions were cured by the treatment of topical steroid and antifungal therapy. However, the disorder was not cured and the symptoms had not improved.

The patient was hospitalized at Razi Hospital when he was brought in for the evaluation of his skin lesions, which were caused by Staphylococcus aurous and septicemia caused by Klebsiella. He was treated with intravenous antibiotic therapy and after a few days his general condition improved, except for his skin lesions. The blood and urine tests were normal but his plasma zinc level was measured to be at 12 μg/dL (normal levels are 50–150 μg/dL). Moreover, microscopic examinations showed Candida albicans on the inguinal area, which was treated in 12 days by the administration of Baflokonazol 6 mg/kg/day. The patient was diagnosed with Acrodermatitis Enteropathica because of his clinical and laboratory test results. He was prescribed 40 mg of oral zinc sulfate per day which resulted in significant improvement of the symptoms within a few days, including the skin lesions. After a month of treatment, all lesions healed, hair began to grow back, and there were no reports of diarrhea. A follow up with the patient was recommended.

The patient was a 25-year-old woman. When she was 2 months old her mother stopped breastfeeding her and skin eruptions covered her entire body. The skin eruptions, such as vesiculobullous and erythematous scaly lesions, started around her mouth and spread throughout her face and legs. She also had diarrhea. Since she had symptoms suggestive of AE, there were further tests done in order to confirm the diagnosis. The diagnosis was confirmed by measurement of the plasma zinc levels which were at 0.2 mg/L (normal levels are between 0.7 and 1.6 mg/L). Her treatment began with administration of four tablets of 40 mg zinc sulfate per day. After 4 days, there was incredible improvement of her skin rashes and diarrhea, but she had developed behavior disorders. During these 25 years she continued her zinc supplementation treatment.

When she was 15 years of age, her treatment was reduced from 220 mg three times daily to twice daily. However, after 2 weeks, skin lesions began to appear around the mouth. When she was 16 she developed severe vomiting; and as a result, her treatment was increased to 220 mg of spansule-zinc supplements three times a day again. Afterwards, her plasma zinc was decreased from 1.1 to 0.3 mg/L and she suffered from rashes, similar to eczema and mild seborrheic dermatitis, on the sides of her nose and rashes similar to acne on her body. These rashes began to disappear 3 weeks after the increasing of spansule-zinc supplements to three times a day: plasma zinc increased to 1 mg/L.

Plasma zinc levels were measured at regular intervals of 6 months. When the patient reached the age of 20, her plasma zinc level decreased to the lowest normal level: 0.7 mg/L. The spnasule-zinc treatment was increased to 10 times a day. Fortunately, the patient’s body was able to tolerate 10 pills per day and her plasma zinc level was maintained at a normal level of 1.4 mg/L.