Anetoderma: causes, symptoms, diagnosis, treatment

Anetodermia (synonym: spotted skin atrophy) is a type of skin atrophy characterized by the absence of elastic tissue.

Causes and pathogenesis of the disease have not been fully established. There are data on the causative role of endocrine and neural disorders. It indicates the influence of neuroendocrine effects. The role of infection (spirochetes) is not excluded, as evidenced by cases of the development of the disease after tick bites. A good therapeutic effect of penicillin therapy allowed some authors to propose an infectious theory of the onset of the disease. Histochemical studies have established that the onset of anethodermia is probably due to the release of elastase from the cells of the inflammation focus that elastolyses.

Symptoms of atrophy of the skin spotted (an anhedodermia). Clinically, several variants are distinguished: foci of atrophy, arising at the preceding erythematous stage (Jadasson-Tibiere type), in the place of urticarachnoe elements (Pellizari type) and on clinically unchanged skin (Schwenninger-Buzzi type). Different variants can be for the same patient. Foci of atrophy can be located on any part of the skin, more often on the upper half of the trunk, on the hands and face, they are small, on average 1-2 cm in diameter, round or oval, white-blue, shiny wrinkled surface. Some elements are herniated like a hernia, pressing them with a finger causes a feeling of falling into the void, while other elements, on the contrary, sink. Anetoderma is an integral part of the Bleggad-Haxthausen syndrome (atrophic spots, blue sclera, fragility of bones, cataracts).

The disease occurs more often in women aged 20-40 years, more often in the central part of Europe. This is apparently associated, in some cases, with the association of an anodol- ogy with chronic atrophic acrodermatitis caused by Br. Burdorferi.

Clinically, several variants of anethodermia are distinguished: foci of atrophy, arising after the previous erythematous stage (classical type of Jatsasson); on externally unchanged skin (type of Schwenninger-Buzzi) and on the site of urtikarno-edematous elements (Pellizari type).

In the classical type of Jadasson's anethoderma, single or multiple spots of irregular oval or round shape appear, up to 0.5-1 cm in diameter, pink or yellowish-pink in color. Elements of lesion are localized more often on the trunk, upper and lower extremities, neck and face, but it is possible to damage the skin of other areas. Palms and soles are usually free from rashes. The stain increases in size and within 1-2 weeks its size reaches 2-3 cm. Erythematous plaques and even nodes of large sizes are described. Gradually, without any subjective sensations on the spot of the erythematous spot, atrophy develops, which originates in the center of the spot. The skin on these places becomes pale, wrinkled, resembling a crumpled tissue paper; The focus slightly protrudes above the level of the surrounding skin in the form of a soft hernial-like protrusion of the skin. When you press your finger on this area of the skin an impression of emptiness appears (the finger falls "deep"). Hence the name of the disease: anetos - emptiness.

With Schwenninger-Buzzi anemodermia, there are also herniated bulging atrophic spots on the back and upper limbs. However, unlike the classical type of Jadasson's anethodermia, the foci of atrophy are much more prominent over the surrounding skin, their surface may have telangiectasia and the first inflammatory stage is always absent.

With the urticar type, the anethodermia develops on the site of blisters, there are no subjective sensations. When pressing on the element, the finger falls into the void.

With all types of anethodermia, a sharp thinning of the epidermis, a complete disappearance of the elastic fibers and dystrophic changes in collagen fibers are noted in the focus of atrophy.

Distinguish between primary and secondary anethodermia. The cause of primary anethodermia is not known. However, it is often combined with such diseases as scleroderma, hypocomplexemia, HIV infection, etc. Secondary anethodermia occurs after the resolution of spotted and papular elements in secondary syphilis, lupus erythematosus, leprosy, sarcoidosis, vulgar acne, and others.

A premature anethodermia (anetoderma prematura) is described that develops in babies born prematurely. The development of this variety is explained by chemical, metabolic changes in the fetal skin. A case of fetal anethodermia in an intrauterine life is described, when the mother suffered from intrauterine borelliosis. This case indicates the existence of congenital anethodermia.

Pathomorphology. In the initial (inflammatory) stage, histological changes are non-specific and are characterized by the presence in the dermis of perivascular infiltrates consisting of lymphocytes and neutrophilic granulocytes. In older elements, one can see atrophy of the epidermis, a decrease in infiltration in the dermis and dystrophic changes in collagen fibers (atrophy stage). This disease is characterized by focal or total absence of elastic fibers. The epidermis, as a rule, is thin. In the electron-microscopic study of lesions of the skin, changes in elastic fibers were observed in the form of sharp thinning and a decrease in their number. According to these authors, the remaining thin elastic fibers consist of a centrally located amorphous substance of low electron density without fibrils, but with their presence along the periphery of the fiber. Detect coarse fibrillar masses, in which microfibrils are sometimes found, sometimes having the form of tubules. In some fibers, there is vacuolar dystrophy. Collagen fibers are not changed. Most fibroblasts with signs of activation of the synthetic function. Macrophages alternate with lymphocytes, among which are the remains of dead macrophages, sometimes here there are plasmocytes and individual tissue basophils. J. Pierre et al. (1984) believe that the presence of fine fibers indicates a new synthesis of elastic fibers after elastolysis, which takes place in this disease.

Histogenesis. The sharp decrease in elastic fibers in the lesions is explained, on the one hand, by the possibility of a primary molecular defect consisting in a decrease in the synthesis of elastin or a microfibrillar component of elastic fibers or a disruption in the formation of desmosin; on the other hand, it is not excluded that the destruction of elastic fibers is caused by elastase released from the cells of the inflammatory infiltrate, primarily such as neutrophilic granulocytes and macrophagocytes. The possibility of increased elastolysis is indicated, in particular by increasing the elastase / antielastase ratio, as noted in other diseases, which are based on the lesion of the elastic fibers. There is evidence to support immune mechanisms in the pathogenesis of anemodermia, as indicated by the frequent detection in infiltrates of plasma cells and a large number of T-lymphocytes with the predominance of T-helper cells among them, as well as signs of leukoclastic vasculitis with perivascular deposits of IgG, IgM and C3-component complement. Given the development of patchy skin atrophy in such different conditions as pigmentary urticaria, xanthomatosis, prolonged use of corticosteroids, penicillin, regression of many dermatoses (tertiary syphilis, tuberculosis, leprosy), it can be assumed that an anodoldering is a heterogeneous state, the basis of which is the death of elastic fibers under the influence of a variety of reasons. In addition, there are data indicating the existence of not only cutaneous forms of anethodermia, but also lesions of other organs, which is confirmed by the combination of anethodermia with cutis laxa.

Treatment of atrophy of skin spotted (an anhedodermia). Penicillin and antifibrinolytic (aminocaproic acid) agents and restorative (vitamins, biostimulants) preparations are recommended.

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