Enteropathic acrodermatitis: causes, symptoms, diagnosis, treatment

Acrodermatitis enteropathica (synonym: Danbolt-Kloss syndrome) is a rare systemic disease caused by zinc deficiency in the body due to its absorption in the small intestine. It is characterized by the presence of erythematous, vesicular and blistering rashes on the skin, hair loss and diabetes. The disease is autosomal recessive, X-linked transmission is possible.

X-linked inheritance (self-healing enteropathic acrodermatitis) and sporadic cases are possible. Zinc deficiency in the body (plasma concentration significantly lower than 68-112 μg/dl) leads to immune disorders, in particular to decreased activity of T-killers, defective delayed-type hypersensitivity reactions, and thymus atrophy. Impaired keratinization is associated with the presence of zinc-dependent enzyme systems in keratinosomes. In self-healing enteropathic acrodermatitis, a decrease in the zinc content in breast milk is noted due to a change in the genetic mechanism of its regulation in the mother. Sporadic cases are possible due to secondary zinc deficiency due to its deficiency in food products, long-term parenteral nutrition, impaired absorption or loss of zinc in inflammatory diseases of the gastrointestinal 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 (adrenal cortex insufficiency, diabetes, hypo- or hyperparathyroidism) disorders, changes in cellular and humoral immunity. These changes lead to a decrease in the activity of T-killers, a change in the delayed-type hypersensitivity reaction and thymus atrophy. However, clinical manifestations of enteropathic acrodermatitis can develop with a lack of zinc in food products, long-term parenteral nutrition, inflammatory diseases of the gastrointestinal tract, alcoholic cirrhosis.

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

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

Differential diagnosis should be made with psoriasis, bullous epidermolysis, hereditary pemphigus, childhood eczema.

Symptoms of enteropathic acrodermatitis. Clinically manifested by skin rashes, alopecia, diarrhea and photophobia. The rashes are predominantly erythemato-pustular in nature, located periorificially, on the hands, feet, as well as in the area of large joints and in skin folds. When they merge, fairly sharply defined foci arise, which take on a psoriasiform appearance due to the abundant layering of scales and scaly crusts. Other symptoms may also be observed: 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, weeping, erosions covered with serous and serous-purulent crusts and scaly crusts may be observed. This clinical picture is very similar to psoriatic lesions. At the same time, there are lesions of the mucous membranes (blepharitis, conjunctivitis with photophobia, glossitis, stomatitis, etc.), hair loss (to the extent of total baldness), dystrophic changes in the nails, paronychia. Patients experience severe diarrhea, exhaustion, mental development disorders, they become tearful and irritable.

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

Treatment of enteropathic acrodermatitis should be comprehensive. Zinc oxide (0.03-0.15 g per day depending on age), a complex of vitamins (groups B, A, C, E) with microelements (zinc, copper, iron, etc.), immunocorrective agents, enteroseptol, externally - softening creams, corticosteroid ointments are used. Patients should follow diet therapy. They should eat more products containing a large amount of zinc (fish, meat, eggs, milk, etc.).

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