Mahboobeh Shokrizadeh - Resident, Oral and Dental Research Center AND Department of Pediatric Dentistry, School of Dentistry, Kerman University of Medical Sciences, Kerman, Iran
Fatemeh Sadat Sajadi - Associate Professor, Oral and Dental Research Center AND Department of Pediatric Dentistry, School of Dentistry, Kerman University of Medical Sciences, Kerman, Iran

BACKGROUND AND AIM: Acrodermatitis enteropathica (AE) is a rare and severe genetic disorder with autosomal recessive inheritance, which is usually diagnosed with deficiency of zinc intestinal absorption. This disease is classified into hereditary and acquired forms that the hereditary form is much rare. AE can be characterized by triad of periorificial and acral dermatitis, alopecia, and diarrhea. The present study aimed to report the dentistry treatment of two boys with hereditary AE and special considerations about this disease. CASE REPORT: Patients included two brothers aged ۶ and ۸ years with AE, and they were examined at a health center. Three days after the birth, the erythematous, erosive, and crusted lesions were seen on their mouths, perianal areas, feet, and hands. The serum zinc levels were normal. The preventive treatment like fissure sealant on four first permanent molars was performed on the ۸-year-old patient, and for the other patient, two mandibular deciduous anterior teeth were extracted. CONCLUSION: AE is a rare genetic disease which can be along with dental anomaly and oral infections. Despite the fact that there are cases with normal serum, the clinical and radiographical examinations will be helpful for the final diagnosis. This study presented two male siblings with normal zinc serum who demonstrated hair and skin lesions without dental disorders.

Acrodermatitis Enteropathica, Oral Manifestations, Child, Case Reports