Idiopathic progressive skin atrophy

Idiopathic progressive skin atrophy (synonyms: chronic atrophic acrodermatitis, chronic atrophic acrodermatitis of Herkeheimer-Hartmann, Pick's erythromyelia) is a disease that is a chronic slowly progressive diffuse atrophy of the skin, mainly of the extensor surface of the extremities, developing in the late stage of borreliosis.

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Causes idiopathic progressive skin atrophy.

Many dermatologists suggest an infectious theory for the origin of idiopathic progressive skin atrophy. The effectiveness of penicillin, the development of the disease after a tick bite, and positive vaccinations of pathological material from patients to healthy people confirm the infectious nature of the dermatosis.

The causative agent is a spirochete of the genus Borrelia. The detection of antibodies (mainly IgG, less often IgM) to the causative agent of chronic migratory erythema in patients with acrodermatitis served as the 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 B. Wilske et al. (1985). The atrophic process can begin several years after the tick bite, the causative agent continues to persist in the skin at the stage of atrophic changes.

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Risk factors

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

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Pathogenesis

Histologically, the first stage reveals thinning and swelling of the epidermis, vasodilation, perivascular infiltration of lymphocytes and histocytes, plasma cells, in the upper part of the dermis - strip-like, sometimes separated from the epidermis by a zone of unchanged connective tissue. In the atrophy stage, thinning of all skin layers, atrophy of hair follicles, sebaceous glands or their absence, destruction or disappearance of elastic and collagen fibers are observed.

Pathomorphology

In the early stage of the process, an inflammatory reaction is observed, expressed in the appearance of a strip-like infiltrate near the epidermis, separated from the latter by a narrow strip of unchanged collagen, and in the deeper parts of the dermis, perivascular infiltrates are formed, consisting of lymphocytes and histiocytes. Then atrophic changes appear, expressed in atrophy of the epidermis and smoothing of its epidermal outgrowths, thinning of the dermis, edema with loosening of collagen fibers with a decrease in their number and focal, mainly perivascular, inflammatory infiltrates. Later, a sharp thinning of the dermis is noted, which occupies only 1/2 or 1/3 of the thickness of normal skin, it is mixed with adipose tissue. Hair follicles are sharply atrophic or absent, sweat glands are involved in the atrophic process much later. Vessels, as a rule. dilated, especially in the deeper parts of the dermis, their walls are thickened, in the superficial parts of the dermis the number of vessels is sharply reduced, there are signs of their obliteration. The subcutaneous fat layer is also subject to atrophy.

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

This disease is differentiated from the early stage of atrophic stripes of the skin (striae distensae). However, in the latter, characteristic changes in the elastic fibers are observed: their disappearance and weak coloration according to the Weigert method. Histological changes in the foci of fibrous thickening of the dermis are difficult to distinguish from scleroderma.

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Symptoms idiopathic progressive skin atrophy.

Clinically, there are initial (inflammatory) and late (atrophic and sclerotic) stages. Early clinical manifestations are swelling, moderate bluish-red erythema, and slight skin infiltration. Strip-like erythema may be observed on the skin of the extremities. Gradually, over several weeks or months, the inflammatory process subsides and the stage of atrophic changes begins. The skin becomes thinner, dry, wrinkled, loses elasticity, and resembles crumpled tissue paper (Pospelov's symptom). Vessels are visible through the thinned skin; tendons are also visible with pronounced atrophic changes. Slight peeling, spotty or diffuse hyperpigmentation alternating with areas of depigmentation are possible. Sebum and sweating are reduced, hair falls out. Atrophic changes similar in clinical picture to anetoderma may be observed on the periphery of foci. During this period, spica-shaped or focal scleroderma-like compactions may develop. Linear compactions are usually located along the ulna and tibia, focal compactions are located near joints, on the dorsum of the feet. Complications may include calcification, amyloidosis, skin lymphoplasia, ulceration, squamous cell carcinoma, contractures due to perivascular fibrous and strip-shaped compactions, and bone changes.

The following stages of the disease are distinguished: initial, inflammatory, atrophic and sclerotic. For many patients, the disease develops unnoticed, since prodromal subjective sensations are not noted. In the first stage, swelling and reddening of the skin with unclear boundaries appear on the extensor surfaces, especially on the trunk and, rarely, on the face. Red lesions with a pinkish or bluish tint may be diffuse or focal. Over time, doughy infiltration develops. Sometimes it is not palpable. The lesions grow along the periphery, forming stripes, with slight peeling on their surface.

Over time (in the course of several weeks or months of treatment), the second stage occurs - atrophic changes. In the areas of infiltration, the skin becomes flabby, thinned, lacking elasticity, dry. As the disease progresses, a rim of hyperemia appears along the periphery of the lesion, muscle and tendon atrophy may be observed. Due to the disruption of skin trophism, sweating decreases, hair falls out.

In 50% of patients, the third - sclerotic stage of the disease is observed. In this case, strip-like or focal scleroderma-like compactions (pseudoscleroderma foci) develop at the site of atrophic changes. Unlike scleroderma, the foci have an inflammatory color with a yellowish tint and translucent vessels are visible on their surface.

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

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Differential diagnosis

In the initial stage, the disease is differentiated from erysipelas, erythromelalgia, acrocyanosis, and in the second and third stages - from scleroderma, lichen sclerosus, and idiopathic Pasini-Pierini atrophy.

Treatment idiopathic progressive skin atrophy.

Penicillin is prescribed at 1,000,000-4,000,000 IU per day, general tonics. Externally, tissue trophism improving agents, softening and vitaminized creams are applied to the atrophic areas.