Idiopathic progressive skin atrophy

Atrophy of the skin idiopathic progressing (synonyms: acrodermatitis chronic atrophic, acrodermatitis chronic atrophic Gerkeimer-Hartmann, erythromyelia Pick) is a disease that is a chronic slow-progressive diffuse atrophy of the skin of the predominantly extensor surface of extremities developing in the late stage of borreliosis.

[1], [2], [3]

Causes of the idiopathic progressive skin atrophy

Many dermatologists suggest an infectious theory of skin atrophy of idiopathic progressing. The efficacy of penicillin, the development of the disease after a tick bite, positive inoculations of pathological material from patients with healthy confirm the infectious nature of dermatosis. AT

The causative agents are spirochaetes of the genus Vorelia. Detection of antibodies in patients with acrodermatitis (mainly IgG, less often IgM) to the causative agent of chronic migratory erythema served as a basis for the conclusion about the commonality of these diseases. However, it is possible that these two diseases are caused by different, but related spirochetes. The heterogeneity of the strains is indicated by Wilske et al. (1985). The atrophic process can begin several years after the tick bite, the pathogen continues to persist in the skin and at the stage of atrophic changes. 

[4], [5]

Risk factors

Trigger factors can be hypothermia, trauma, endocrine and microcirculatory disorders.

[6], [7], [8]

Pathogenesis

Histologically, in the first stage, thinning and edema of the epidermis, vasodilatation, perivascular infiltration from lymphocytes and histocytes, plasma cells, in the upper part of the dermis, are striped, sometimes separated from the epidermis by a zone of unchanged connective tissue. At the stage of atrophy, thinning of all skin layers is observed, atrophy of hair follicles, sebaceous glands or their absence, destruction or disappearance of elastic and collagen fibers.

Pathomorphology

In the early stage of the process, there is an inflammatory reaction, manifested in the appearance of a band-shaped infiltrate near the epidermis, separated from the latter by a narrow band of unaltered collagen, and in the deeper parts of the dermis, perivascular infiltrates consisting of lymphocytes and histiocytes are formed. Then there are atrophic changes, manifested in the atrophy of the epidermis and smoothing of its epidermal outgrowths, thinning of the dermis, puffiness with loosening of collagen fibers with a decrease in their number and focal, mainly perivascular, inflammatory infiltrates. In the future, a sharp thinning of the dermis, which takes only 1/2 or 1/3 of the thickness of normal skin, is noted, it is replaced by fatty tissue. Hair follicles are sharply atrophic or absent, sweat glands in the atrophic process are involved much later. Vessels, as a rule. Expanded, especially in the deeper parts of the dermis, their walls thickened, the number of vessels in the superficial sections of the dermis sharply reduced, there are signs of obliteration. Atrophy is also exposed to the subcutaneous fat layer.

In the foci of compaction, collagen fibers are thickened, homogenized, as in scleroderma, and in fibrous nodules they are hyalineized.

Differentiate this disease from the early stage of atrophic skin bands (striae distensae). However, at the latter, characteristic changes in elastic fibers are observed: their disappearance and weak coloring according to the Weygert method. Histological changes in the foci of fibrous thickening of the dermis are difficult to distinguish from scleroderma.

[9], [10], [11]

Symptoms of the idiopathic progressive skin atrophy

Clinically distinguish between the initial (inflammatory) and late (atrophic and sclerotic) stage. Early clinical manifestations are puffiness, moderate bluish-red erythema, slight infiltration of the skin. There can be a strip-like arrangement of erythema on the skin of the extremities. Gradually, within a few weeks or months, the inflammatory process subsides and the stage of atrophic changes comes. The skin becomes thinner, becomes dry, wrinkled, loses its elasticity, looks like a crumpled tissue paper (Pospelov's symptom). Through the thin skin radiograph vessels, with pronounced atrophic changes visible tendons. There is a slight peeling, spotted or diffuse hyperpigmentation alternating with the areas of depigmentation. Salo and sweating are reduced, hair falls out. At the periphery of the foci may be observed atrophic changes, similar in clinical picture with an antedoderm. During this period, spicate or focal sclerodermiform seals may develop. Linear seals are usually located along the ulnar and tibial bones, focal - near the joints, at the rear of the feet. As a complication, calcification, amyloidosis, lymphoplasia of the skin, ulceration, squamous cell carcinoma, contractures due to vascular fibrous and strip-like seals, bone changes may develop.

Distinguish the following stages of the disease: initial, inflammatory, atrophic and sclerotic. For many patients, the disease develops unnoticed, because prodromal subjective sensations are not observed. In the first stage, on the extensor surfaces, especially on the trunk and, rarely, on the face, puffiness and reddening of the skin with unsharp borders appear. Foci of red color with a pink or cyanotic hue may have a diffuse or focal character. Over time, test infiltration develops. Sometimes she is not probed. Foci of lesion grow along the periphery, forming bands, on their surface there is insignificant peeling.

Over time (in the treatment of several weeks or months) comes the second stage - atrophic changes. In places of infiltration, the skin becomes flabby, thinned, devoid of elasticity, dry. When the disease progresses along the periphery of the focus, a corolla of hyperemia appears, and muscle and tendon atrophy can be noted. Due to violation of trophic skin sweating is reduced, hair falls out.

In 50% of patients there is a third - sclerotic stage of the disease. At the same time, at the site of atrophic changes, stripy or focal scleroderm-like seals develop (pseudoscleroderma foci). In contrast to scleroderma, foci have an inflammatory color with a yellowish tinge and radiating vessels are visible on their surface.

Some patients may have peripheral neuritis, muscle weakness, changes in joints, heart, lymphadenopathy, increased ESR, hyperglobulinemia, sometimes cryoglobulinemia.

[12], [13]

Differential diagnosis

The disease in the initial stage is differentiated from erysipelas, erythromelalgia, acrocyanosis, in the second and third stages - from scleroderma, sclerotrophic lichen, idiopathic atrophy of Pazini-Pierini.

Treatment of the idiopathic progressive skin atrophy

Prescribe penicillin at 1000000-4000000 units per day, fortifying drugs. Outwardly, at sites of atrophy, funds are added that improve trophic tissue, softening and vitaminized creams.