Atrophoderma is a variant of skin atrophy, which includes a number of diseases based on degenerative–atrophic processes in the epidermis and dermis. The disease has a tendency to a protracted course, has no gender component, is off-season. The clinic of each pathology included in the group of atrophoderma is specific; a common feature is the formation of atrophic foci on the skin. In the diagnosis, the histological examination of the skin biopsy is crucial. Treatment is symptomatic (vitamin therapy, physiotherapy and balneotherapy), the prognosis is favorable. There is an oncological vector during atrophoderma.
Atrophoderma is a few rare chronic pathologies that are based on a complex of dystrophic skin changes that occupy an intermediate position between classical skin atrophy and classical scleroderma. They can be congenital and acquired, symptomatic or act as an independent disease. The causes and mechanism of development of atrophoderma have been the subject of dermatology research for a century, but are not fully elucidated today. For example, wormlike atrophoderma was described at the end of the XIX century when studying follicular keratosis of the eyebrows (brow erythema Unna), as the outcome of this disease. Reticular atrophoderma has been known as Jacobi’s poikiloderma since 1906 (Muller’s reticular erythematous atrophoderma, Milian-Peren’s erythematous—atrophic poikiloderma, Gluck’s atrophic dermatitis, Thomson’s syndrome, Rotmund syndrome). Limited Pasini-Pierini atrophoderma was first described in 1875, and neurotic atrophoderma – in 1864 by the English surgeon Paget.
The clinical picture of atrophoderma in each individual case has its own characteristics. Since the exact etiology of the disease has not been established, treatment is symptomatic. The relevance is associated with the possibility of the degeneration of the poikilodermic process into a malignant neoplasm.
Atrophoderma occurs due to a violation of nutrition of all layers of the skin as a result of unspecified causes. Dermatologists believe that the basis of reticular atrophoderma (poikiloderma) are vascular disorders – desolation, narrowing of the lumen of the vessels feeding the skin – of a neuroendocrine or infectious-toxic nature (radiation exposure, UVI, systemic dermatoses).
Worm-like atrophoderma appears due to changes in the trophic structure of the skin, the destruction of its glands due to endocrine-autonomic disorders (violation of vitamin A synthesis, diabetes mellitus). Neurotic atrophoderma is the result of disorders in the central and peripheral nervous systems, leading to a disorder of metabolic processes (polyneuritis, nerve damage). Limited Pasini-Pierini atrophoderma is based on a combination of all of the above causes against the background of a sharp weakening of general and local immunity.
Classification of atrophoderma is rather a quantitative interpretation of the diseases included in its group. There are:
- Worm–like atrophoderma is a disease of the young, the main element is a follicular plug symmetrically located on the cheeks with an outcome in a small atrophic scar. Such a mesh “pitted” skin resembles the bark of a tree or honeycomb.
- Atrophoderma idiopathic Pasini-Pierini is a rare disease that affects mainly young women. Localized on the trunk. The primary element is an oval spot sinking in the center, which transforms into a plaque, and then into a limited focus of atrophy.
- Neurotic atrophoderma – the skin of the extremities is affected, the trophic tissues are disturbed with the appearance of foci of atrophic dystrophy, thinning of nails and hair loss.
- Atrophoderma reticulata (poikiloderma) is a disease beyond age and gender. It is localized most often in open areas of the skin in the form of erythema with an outcome in a pigmented focus of atrophy.
Symptoms of atrophoderma
The clinic of atrophoderma is individual for each disease included in the group of this pathology. Worm-like atrophoderma has the most characteristic clinical picture: young people have symmetrically arranged small (1-3 mm), sinking skin defects of bizarre outlines up to 1 mm deep on their cheeks. These are deformed follicles filled, as a rule, with sebaceous plugs. They merge, form intersecting “waves”, “lines”, “cells”, which, together with the areas of normal skin between them, resemble a pattern of tree bark eaten by worms, or honeycombs. With age, the symptoms are smoothed out.
The clinic of the idiopathic form resembles a limited focus of superficial scleroderma, it is pressed into the surrounding healthy skin, small in size (up to 2 cm), oval in shape, soft on palpation, flesh-colored, single or multiple, tends to merge with the formation of scalloped forms. It is located on the stomach or back.
The neurotic form is characterized by a lesion of the fingers and toes: the skin is glossy, tense, it seems that when the hand or foot is bent, it will “burst”, the skin becomes bluish, as in acrocyanosis, due to insufficient capillary blood supply. Over time, the nutrition of this area of the skin practically stops, it becomes deathly pale, becomes thinner, transparent nail plates and hair loss are observed in the focus of atrophy.
Poikiloderma, or mesh atrophoderma, is located on open areas of the skin, in the decollete area. Visually it looks like a mottled skin surface due to the alternation of atrophic, pigmented spots with depigmented ones. Possible transformation into melasma, fungal mycosis.
Diagnosis and treatment
Atrophoderma is diagnosed on the basis of clinical manifestations and the results of histological studies. The morphological picture of each pathology is individual. Differentiate the disease with parapsoriasis, scleroderma, dermatomyositis, radiation dermatitis, fungal mycosis, melanosis, keratosis of Siemens, Bazex syndrome, brow ulcer, chickenpox, vulgar acne. The basis of differential diagnosis is the clinic in combination with the results of a skin biopsy.
Treatment consists in the rehabilitation of foci of chronic infection, primarily tuberculosis, syphilis. Effective therapy of long-term somatic diseases (diabetes mellitus, thyroid and parathyroid gland diseases, rheumatism, obesity) is necessary. Symptomatically, vitamin therapy is used (vitamin A, externally and internally, multivitamins), drugs that improve tissue trophism (dexpanthenol), iron preparations, diazepam. Thermal procedures, dermabrasion, bran baths, sea baths, radon baths are effective; mud therapy (Crimea, Naftalan), massage and physical education to improve skin nutrition.
Prevention of atrophoderma is the priority of a healthy lifestyle. The prognosis is favorable for life. With poikiloderma, mandatory observation by a dermatologist is necessary due to the possibility of degeneration into reticulosarcomatosis.