Enteropathic acrodermatitis (Dunbolt-Kloss disease)

Enteropathic acrodermatitis - a disease associated with a violation of zinc absorption, inherited in an autosomal recessive type. As a result of a defect in the proximal parts of the small intestine, the formation of more than 200 enzymes is disrupted. Dysplasia and cell division are dominant; suffer from the gastrointestinal tract (atrophy of the intestinal mucosa, secondary decrease in the activity of disaccharidases), the immune system (lymphopenia, impaired T-cell differentiation, decreased antibody production).

ICD-10 code

E83.2. Violations of the exchange of zinc.

Symptoms

Enteropathic acrodermatitis manifests itself already with the 2-3 nd life of life with the abolition of breast milk, the early onset of artificial feeding. There is a frequent watery stool, anorexia develops, weight gain is reduced. The increased nervous reflex excitability is characteristic. Changes in the skin in the form of a symmetrical rash around the mouth, nasal passages, behind the ears, on the distal parts of the limbs appear gradually: at the beginning of the rash, the erythematous character is born, then bullae, vesicles, pustules, hyperkeratosis are formed. When the mucous membranes are damaged, gingivitis, stomatitis, glossitis, blepharitis, conjunctivitis develop. On the immunodeficient background, a secondary infection quickly joins.

Diagnostics

Diagnosis of enteropathic acrodermatitis is based on the evaluation of the clinical picture, the detection of a decrease in zinc in the blood serum, excretion of zinc in the urine, absorption of ⁶⁵ Zn. In the biochemical analysis of blood, a decrease in the activity of alkaline phosphatase, an increase in the content of ammonium, a decrease in the concentration of beta-lipoproteins, a change in the immune status are noted. Histological examination of the mucous membrane allows detecting characteristic inclusions in the Pannet cells. The disease is differentiated with a secondary impairment of zinc absorption in inflammatory bowel diseases, mucosal atrophy, post-resection syndrome.

Treatment

Assign sulfate, acetate or zinc gluconate for 10-20 mg per day for children of the first year of life, for patients over the year the daily dose is 50-150 mg, depending on the age.