Enteropathic acrodermatitis: causes, symptoms, diagnosis, treatment

Acrodermatitis enteropathic (synonym: Dunbolt-Kloss syndrome) is a rare systemic disease caused by a deficiency of zinc in the body due to its absorption in the small intestine. It is characterized by the presence on the skin of erythematous, bubble and bladder rashes, hair loss and diabetes. The disease is autosomal-reciprocal, an X-linked transmission type is possible.

Possible X-linked type of inheritance (self-healing enteropathic acrodermatitis) and sporadic cases. Zinc deficiency in the body (plasma concentration is well below 68-112 μg / dL) leads to immune disorders, in particular to a decrease in T-killer activity, inferior delayed hypersensitivity reactions, and thymic atrophy. The violation of keratinization is associated with the presence of zinc-dependent enzyme systems in keratinosomes. With self-healing enteropathic acrodermatitis, there is a decrease in zinc in breast milk due to a change in the genetic mechanism of its regulation in the mother. Sporadic cases are possible because of secondary deficiency of zinc with a lack of it in food, with prolonged parenteral nutrition, impaired absorption or loss of zinc in inflammatory diseases of the digestive tract, alcoholic cirrhosis.

Causes and pathogenesis of enteropathic acrodermatitis. It is believed that the development of the disease is associated with a genetically determined violation of zinc absorption by the intestine, endocrine (insufficiency of the adrenal cortex, diabetes, hypo- or hyperparathyroidism) disorders, changes in cellular and humoral immunity. These changes lead to a decrease in the activity of T-killers. Change in the reaction of delayed hypersensitivity and atrophy of the thymus. However, clinical manifestations of acrodermatitis enteropathic can develop with a lack of zinc in food, prolonged parenteral nutrition, inflammatory diseases of the gastrointestinal tract, alcoholic cirrhosis.

Histopathology. In the epidermis there are phenomena of acantholysis, intercellular edema with the formation of suprabasal blisters, in the dermis - a picture of nonspecific dermatitis with lymphohistiocytic infiltration around the vessels.

Pathomorphology. The histological picture is nonspecific, it is an eczematoid reaction. In the acute period there is a slight thickening of the epidermis with partial exfoliation of the stratum corneum, focal parakeratosis, intercellular edema with the formation of suprabasal blisters, sometimes containing acantholytic cells. Necrotic changes in the epidermis may occur. In the dermis - edema, perivascular lymphohistiocytic infiltrates, localized mainly in the upper part of the dermis. In the chronic period of the process psoriatic form of acanthosis, massive hyperkeratosis with focal parakeratosis, weakly expressed spongiosis are observed. However, the inflammatory reaction in the dermis is similar to that in the acute period.

Differential diagnosis should be carried out with psoriasis, bullous epidermolysis, hereditary pemphigus, children's eczema.

Symptoms of enteropathic acrodermatitis. Clinically manifested by skin rashes, alopecia, diarrhea and photophobia. Eruptions of mainly erythematous-pustular nature are arranged periodically, on hands, feet, and also in the area of large joints and in folds of the skin. When they merge, there are quite sharply outlined foci, taking psoriatic form due to a profuse layering of scales and scaly-crusts. There may be other symptoms: nail dystrophy, growth retardation, stomatitis, glossitis, mental disorders. The disease usually begins in early childhood. The clinical picture is characterized by simultaneous lesions of the skin, gastrointestinal tract (diarrhea) and sometimes hair loss. In the lesions can be observed mocculation, erosion, covered with serous and serous-purulent crusts and scaly crusts. This clinical picture is very similar to psoriatic foci. At the same time, mucosal lesions are observed (blepharitis, conjunctivitis with photophobia, glossitis, stomatitis, etc.), hair loss (to the degree of total alopecia), dystrophic nail changes, paronychia. Patients with marked diarrhea, exhaustion, mental development disorders, they become whiny, irritable.

The prognosis of the disease is not very favorable; patients often die from concomitant diseases.

Treatment of enteropathic acrodermatitis should be complex. Apply zinc oxide (0,03-0,15 g per day, depending on age), a complex of vitamins (group B, A, C, E) with trace elements (zinc, copper, iron, etc.), immunocorrecting agents, enteroseptol , externally - softening creams, corticoid steroid ointments. Patients should comply with diet therapy. They should eat more foods containing a large amount of zinc (fish, meat, eggs, milk, etc.).

[1], [2], [3], [4], [5], [6]