Pseudo-syringomyelic acropathy is a rare chronic systemic disease of unclear etiology, the skin manifestations of which are based on dystrophic changes. Usually affects the lower extremities. It is clinically manifested by the formation of ulcers on the plantar surface of the feet against the background of loss of temperature sensitivity, hyperhidrosis, onychodystrophy, ankylosing joints and mutation. Pain and tactile sensitivity are preserved. The disease is diagnosed on the basis of anamnesis and a complete clinical and laboratory examination of the patient. Vasoactive drugs are used, local remedies are prescribed, hyperbaric oxygenation and deep radiotherapy are used, sympathectomy is performed. There is a low effectiveness of therapeutic measures.
General information
Pseudo-syringomyelic acropathy is a rare pathological process of a systemic nature, accompanied by the formation of trophic ulcers on the skin of the extremities. The disease exists in two forms: hereditary family pathology (Peron-Drocke-Coulomb syndrome), first described in 1942, and acquired non-family pathology (Bureau-Barrier-Tom acropathy), the first description of which was compiled in 1953. From a clinical point of view, ulcerative defects of familial and non-familial acropathy proceed identically.
Pseudo-syringomyelic acropathy has a bright gender coloring, usually men over 40 years old suffer. The disease occurs at any time of the year, but worsens in winter, does not have endemic. There are no data on the proportion of dermatological diseases in the structure (probably due to the rarity of pathology). The urgency of the problem is determined by the complications of pseudo-syringomyelic acropathy, which significantly reduce the quality of life of patients, a high frequency of diagnostic errors due to the sporadic nature of the pathology, as well as the possibility of degeneration into squamous cell skin cancer with prolonged absence of therapy.
Causes and classification
In modern dermatology, there is a division of pseudo-syringomyelic acropathy into two variants. Distinguish:
- Congenital familial form – genodermatosis, which is characterized by a disorder of all types of sensitivity on the legs and trophic disorders caused by underdevelopment of the posterior and lateral pillars of the lower part of the spinal cord.
- Acquired non-familial form, manifested by trophic ulcers of the skin of the lower extremities against the background of chronic alcoholic polyneuropathy.
The etiology of the familial form of the disease is clear – it is a genodermatosis inherited by recessive or dominant type and combined with other congenital anomalies. The cause of the development and pathogenesis of the acquired form of the disease have not been fully clarified. Most dermatologists, giving priority in the development of the pathological process to changes in the autonomic nervous system and chronic alcohol intoxication of nerve cells, believe that the triggers of the skin process in this pathology are exogenous factors (hypothermia of the skin, injuries, wearing uncomfortable tight shoes) against the background of constantly existing endogenous causes (toxic liver diseases, vitamin deficiency, metabolic disorders).
The mechanism of development is a violation of the trophic skin as a result of alcoholic polyneuropathy. In patients suffering from chronic alcoholism, as a result of the toxic effect of alcohol on the nerve endings, metabolic disorders occur in the peripheral and central nervous system, which leads to malfunctions of the autonomic nervous system. With alcoholic neuropathy, the sensory function of the peripheral nerves is disrupted, as a result, the patient begins to perversely feel the ambient temperature, injuries, scuffs on his feet from uncomfortable shoes, etc.
The process is aggravated by a lack of vitamin B1 caused by pathological changes in the digestive tract. Since B1 is an antioxidant involved in the transmission of nerve impulses, its low values lead to irreversible trophic changes. In parallel, in the body of patients who often have unfavorable heredity for a number of enzymes, there is an accumulation of acetaldehyde – an intermediate product of the breakdown of ethanol, which finally destroys the nerve endings.
Symptoms
With congenital pathology, deformities in the form of bone deformity, a decrease in the size of the foot and spinal defects predominate. Visceral pathology is possible in the absence of vegetative disorders. Neurologically, the preservation of only deep sensitivity is noted. The skin and bone manifestations of congenital and acquired pathology are identical.
Pseudosiryngomyelitic non-familial acropathy begins with the appearance of foci of plantar hyperkeratosis on both legs at the foot support points against the background of hyperhidrosis. The focus of hyperkeratosis becomes diffuse over time, a reddish rim appears on the border between healthy and affected skin. There are verrucose growths. The back of the foot is never involved in the process. The toes become like “drumsticks”, the nails take the form of “watch glasses”.
Due to trophic disorders and bacterial lesions caused by excessive sweating, ellipsoid painless deep ulceration of a bluish hue with dense smooth edges and a necrotic bottom are formed on the soles. The discharge of ulcers is purulent, with deep penetration into the skin (to the bone) – with bone sequestration. Over time, ulceration is joined by swelling of the foot as a result of a violation of sympathetic innervation, cyanosis and osteolysis of the distal parts.
The foot is deformed, movements in the joints are limited. Root sensitivity is disturbed, Achilles reflexes disappear. Temperature and pain sensitivity are preserved, muscle tone is reduced. Thus, a triad of symptoms characteristic of pseudo-syringomyelic acropathy is formed: sluggish acro-ulceration, osteoporosis and destruction of the bones of the feet, polyneuropathy with partial or complete loss of temperature sensitivity.
Diagnostics
The clinical diagnosis is made by a dermatologist on the basis of anamnesis, characteristic symptoms of pseudo-syringomyelic acropathy, the results of morphological studies and X-ray examination data, during which the phenomena of progressive arthritis, “creeping” of bones on top of each other, dislocations, fractures and areas of destruction of the bones of the feet are detected. Histologically, there is the development of fibrosis in the sympathetic nodes of the lumbar spine and disorders in the posterior spinal cord.
Pseudo-syringomyelic acropathy is differentiated with syringomyelia, spinal dryness, diabetic macroangiopathy and polyneuropathy, leprosy, obliterating endarteritis, acrosclerotic form of scleroderma and neural amyotrophy.
Treatment
Patients with pseudo-syringomyelic acropathy should be treated collectively, with the participation of not only a dermatologist, but also a neurologist, surgeon, orthopedist, gastroenterologist, rehabilitologist, physiotherapist and radiologist. Unfortunately, there are no sufficiently effective methods of therapy at the present stage, the pathological process is characterized by high resistance to symptomatic therapy. In case of exacerbations, hospitalization with immobilization of the affected limb is indicated.
Antihypertensive and antibacterial agents, ganglioblockers, sympatholytics, endothelioprotectors, antioxidants, drugs to improve blood microcirculation, adaptogens, vitamins B, A and E. Novocaine paravertebral blockades of the lumbosacral spine are used in the treatment of pseudo-syringomyelic acropathy, sympathectomy, sequestration removal if necessary. There is a positive effect of hyperbaric oxygenation.
Antiseptic dressings, solutions of aniline dyes and anti-inflammatory ointments are applied topically. Long-term non-healing ulcers with mutation of the distal phalanges of the toes are subject to surgical amputation. The ineffectiveness of complex therapy is an indication for the use of deep radiotherapy on the lumbar sympathetic nodes. Many patients are recommended to use orthopedic shoes.
