Description, Causes and Risk Factors:
Stiffness and tightness of the skin of the fingers, with atrophy of the soft tissue and osteoporosis of the distal phalanges of the hands and feet; a limited form of progressive systemic sclerosis occurring with Raynaud phenomenon and scleroderma of the forearms.
Acrosclerosis is one of those conditions frequently incorrectly diagnosed as Raynaud's disease. True Raynaud's disease consists of symptoms of an intermittent nature. The pallor or cyanosis of the extremities is precipitated by cold, and nutritional changes when they occur are limited to changes in the skin; massive gangrene never occurs. All those diseases which may produce the phenomenon simply as a symptom must be excluded before Raynaud's disease is diagnosed.
The most obvious changes in acrosclerosis are found on the face, hands, and feet. Histologically, edema causes swelling and separation of the collagen bundles of the cutis which eventually become hyalinized. There is cellular infiltration around the vessels, and obliterative sclerosing changes involve the intima. Dermal appendages atrophy.
Most likely this disease is caused by a number of inherited (genetic) abnormalities, which are triggered by environmental factors.
Fetal cell theory and microchimerism.
Triggering the immune response.
Inflammatory response and autoimmunity.
Risk factors may include:
The many complications of acrosclerosis can have a major impact on a person's sense of well-being. Patients are greatly concerned about changes in their appearance, particularly those changes caused by tightening of the facial skin. Depression has great impact, along with pain, on reducing patients' ability to function socially.
The majority of published cases have been those offemales, symptoms beginning before middle age.Severe pain in the fingers is almost always present andthe fingers become stiff and are often blue in colour,but if the hands are raised the skin becomes white andbloodless. Cold aggravates or initiates colour changes.When the patient attempts to warm the fingers theybecome deep red and more painful. As the patient learnsto avoid cold and as the condition advances, the skinof the fingers becomes a chronic dusky red with waxywhite isolated areas at the tips of the fingers. Graduallythe colour changes become permanent and are no longerintermittent. The skin becomes thicker and smootherthan normal. The fingers appear swollen, giving theskin a stretched, tense, shiny appearance. It is impossibleto move the skin, and it seems as if it is adherent to theunderlying bones and joints. Small dark areas of superficial gangrene appear on the fingers, frequently at theside of the nail. These spots are extremely painful andeither heal slowly, leaving scars, often circular, sometimes linear, and sometimes star-shaped, or infectionoccurs and a whitlow develops. Such a whitlow is verytroublesome and chronic, and results in destruction ofsoft tissues, the nails, or the phalanges.
Black hard necrotic tissue often builds up below and atthe edge of the nail, deforming the tip of the finger.Frequently there is chronic infection below this hardenedtissue, and beads of pus may ooze out at the junctionof this tissue and the more normal skin. Touching thenail or the hardened necrotic tissue causes great pain.The nail and part of the finger-tip may come away as aslough, leaving a healing, scarred, and shortened fingeron which no nail regrows or at most a small beakshaped nail appears with loose edges which are crackedand turned upwards. In the centre the nail may haveridges of differing thickness.
There is gradual atrophy and absorption of thephalanges, so that each segment of the finger becomesshorter, and sequestra may be discharged from theshortened end of the finger.
There are no specific tests for acrosclerosis. The doctor may suspect acrosclerosisafter taking a history of the symptoms and performing a physical examination. As part of this examination, the doctor does the following:
Presses affected tendons and joints to detect crackling or grating sensations, which can indicate changes related to scleroderma beneath the skin.
Examines the fingernails underneath a microscope. The doctor may find changes in capillaries that are characteristic of scleroderma or mixed connective tissue disease.
Checks the skin for thickened and hardened areas. The major signs of acrosclerosisare hardening and thickening of the skin in any areas on the fingers and toes.
Although there is still no known treatment for acrosclerosis the underlying process of scleroderma, specific drugs and treatments help combat the various mechanisms and consequences of the disease. Patients should receive treatments for specific complications as early as possible in the course of the disease, to reduce progression before irreversible hardening of tissues occurs.
Many drugs that are useful for other autoimmune inflammatory disorders have not proven to be very effective for acrosclerosis. Experimental work is ongoing to develop procedures or to find drugs that can treat the underlying processes that cause damage.
The disease can evolve slowly over time with few symptoms, or progress rapidly and become very severe. The patient, then, must live with considerable uncertainty and emotional stress. Support associations, non-medical aids to help relieve symptoms, and other lifestyle measures can be extremely important and helpful.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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