What causes significant bumps on the hands
The name scleroderma comes from the ancient Greek words "skleros" (= hard) and "derma" (= skin). It indicates a key symptom, the hardening of the skin. Scleroderma is an autoimmune disease of the connective tissue of unknown cause, which leads to an increase in connective tissue fibers with consequent thickening and hardening of the skin. This process is called "sclerosis". Furthermore, not only the skin, but also internal organs such as the lungs, digestive tract and kidneys can be affected.
Classification of scleroderma
The disease group of scleroderma is generally divided into two subgroups: the so-called "local" and the "systemic" scleroderma. The former is also called "Morphea".
The main difference between the two groups is that "circumscribed" scleroderma only affects the skin, while "systemic" scleroderma can also affect internal organs. In addition to these two main groups, there are also some other diseases that lead to sclerosis of the skin, such as nephrogenic fibrosing dermatopathy, mixed collagenosis, and chronic graft-versus-host disease. These so-called "pseudoscleroderma" are clinically similar to scleroderma, but they are different diseases. Only systemic scleroderma is presented below; the other forms are not discussed in detail here.
Systemic scleroderma is divided into two further subgroups, the "diffuse" and the "limited cutaneous" (= limited to the skin) form. The diffuse form spreads over the entire body, while the limited one is limited to the hands and feet to the wrists and ankles. A subtype of limited cutaneous scleroderma is CREST syndrome. The acronym CREST stands for calcinosis cutis (= skin calcification), Raynaud's phenomenon (= attack-like, painful, cold-induced discoloration due to vascular cramps of the fingers), esophageal movement disorders (esophagus = esophagus), sclerodactyly (= hardening of the fingers) and telangiectasia (= enlarged skin vessels) , which summarizes the main symptoms of the disease. The cause of the two forms of scleroderma is not fully understood, but it is assumed that there is narrowing of the small vessels and overproduction of inelastic, stiff, hard connective tissue fibers.
Systemic scleroderma is a rare disease with a frequency of about 1 in 10,000. Women are about four times more likely to get sick than men. It usually begins between the ages of 30 and 50.
Symptoms of diffuse systemic scleroderma
First of all, it must be emphasized that there can be very different courses in systemic scleroderma. Not everyone will develop all symptoms, and not every symptom appears at the same time and at the onset of the disease. Most of the time, symptoms appear slow, insidious, and there is usually time to begin effective therapy. It is often possible to prevent infestation of the internal organs or the long-term effects.
Initially, there is a slight swelling of the skin, mostly on the fingers, hands and forearms, but possibly also on the torso and face. This is followed by thickening and hardening of the skin (due to increased production of inelastic connective tissue fibers). The skin becomes taut and can no longer be lifted up in folds. Later, the swelling and hardening spreads to the remaining parts of the extremities and trunk. The mobility of the joints under the firm, hard skin is increasingly restricted. The tense skin and blood supply problems can lead to tapering of the nose, ears and phalanxes with nail deformities and fingertip ulcers. The mouth opening becomes smaller as the mouth folds. Pigmentation disorders, enlarged small vessels in the nail folds and on the face, sometimes nail fold bleeding, as well as calcium deposits under the skin can complete the clinical picture. The drying out of the skin and the mucous membranes (conjunctiva, nasal, oral and / or vaginal mucosa) also occasionally occurs.
Raynaud's symptom is the paroxysmal fading of the fingers, which is first followed by a purple-blue, later a red discoloration. This phenomenon is very common even among healthy people, it is observed in about 4% of the total population. In people suffering from scleroderma, however, it occurs much more frequently (in
over 90% of patients), and the number of daily seizures is also significantly higher (greater than 5). It can be triggered by cold, but it can also occur spontaneously. Smoking is general for scleroderma, but is particularly harmful for this symptom.
Esophagus and intestines
The stiff connective tissue fibers can be overproduced not only in the skin, but also in some internal organs. The thickening and hardening of the wall of the digestive organs is the result.
Difficulty swallowing, stabbing pain, heartburn in the middle of the upper abdomen (especially in the first few minutes after eating or lying down), emaciation due to impaired digestion and nutrient absorption, and constipation are possible symptoms.
The overproduction of connective tissue fibers in the lungs can lead to a reduction in the elasticity of the lungs and a lengthening of the gas exchange path. As a result, breathing problems (difficult breathing, usually after physical exertion such as physical work, climbing stairs, jogging, sports) can appear.
Heart and pulmonary arteries
The thickening and hardening of the heart and pulmonary arteries can (as with lung involvement) lead to reduced resilience during physical work. Cardiac arrhythmias can sometimes also be observed.
Kidney involvement manifests as high blood pressure and patients complain of frequent headaches.
Joints, muscles and tendons
Joint pain and restricted mobility due to hardening of the skin are typical. Muscle weakness and sore muscles are also possible. You may feel "friction" over the tendons.
Hoarseness, deformity of the eyelids, cataracts, conjunctivitis, narrowing of the vaginal entrance, and other sexual problems and bone loss can also develop.
Symptoms of Limited Scleroderma
Basically, the symptoms are very similar to those of the diffuse form. The main difference is that in limited scleroderma, the skin of the trunk and face is often spared, and that organ involvement occurs much less frequently and more slowly. On the other hand, calcium deposits under the skin are more common. Diagnosis and therapy are the same for both forms.
Diagnosis of systemic scleroderma
The first diagnostic measures to confirm the diagnosis usually have to be carried out on an inpatient basis, as the decision about a therapy must be made relatively quickly. Later on, the examinations can also be carried out on an outpatient basis.
Autoantibodies in the blood
The so-called antinuclear autoantibodies (ANA), which make it easier for the doctor to classify the disease into the various subtypes, are important for the diagnosis of scleroderma. However, they do not cause any noticeable symptoms.
Other laboratory parameters
Most of the other laboratory parameters are initially in the normal range, changes only show up in the case of organ involvement. Digestive problems can lead to deficiency symptoms (e.g. low vitamin and iron levels in the blood); if the kidneys are involved, increased kidney values and protein are often found in the urine. The blood cell sedimentation and the muscle enzymes are activity markers.
The digestive tract is always examined by radiological imaging using a swallow of porridge, but other methods (e.g. gastroscopy, digestive tests) may also be necessary. The function of the lungs is assessed using lung function tests and x-rays, the heart and pulmonary arteries using EKG, echocardiography, stress tests (ergometry) and possibly in rare cases cardiac catheterization. Kidney involvement can be detected not only with the laboratory parameters already mentioned above, but also by sonography (ultrasound). The degree of narrowing of the finger arteries is determined by oscillometry. Calcium deposits can be detected by soft tissue x-rays.
Therapy of systemic scleroderma
Spontaneous healing without therapy does not occur in scleroderma.
Although a treatment that would eliminate the unknown cause does not exist, most symptoms are treatable. Many therapies are also important in preventing the complications. Since the course of the disease is individually different, there are no uniform schemes. The therapy depends on the individual condition of the patient.
Because of the narrowing of the vessels in general, but especially in the fingers (Raynaud's symptom) and the lungs, vasodilator drugs are necessary in almost all patients, especially in winter when the cold is an additional provoking factor. Treatment with tablets or very effective infusions is possible. The infusion therapies are carried out either as an inpatient or part-time inpatient. The vasodilation slows down the course of the disease and is therefore also essential for prevention. These drugs are particularly important for the healing of finger ulcers. Examples are calcium channel blockers, prostaglandin derivatives and endothelin receptor antagonists.
Anti-inflammatory therapy is sometimes also required (e.g. for muscle and joint problems). B. cortisone preparations, cyclophosphamide, azathioprine, etc. prescribed.
If the skin hardens very quickly, methotrexate or light therapy with UV light are usually used.
If the kidneys are involved, so-called ACE inhibitors are often administered, if the heart is involved, medication for arrhythmias is administered, and acid blockers are used in the case of problems with the salivary duct.
Supplementary physiotherapy is also very important: regular lymph drainage, physiotherapy, finger movement exercises, underwater massages, breathing exercises, warm thermal baths are recommended (depending on the individual condition).
Your attending doctor will select the most suitable for you from the above treatments depending on your current and individual condition.
Prevention of progression and complications
Smoking is generally one of the body's greatest enemies, but in systemic scleroderma its deleterious effects are extremely serious as it further narrows the vessels and maintains a generalized inflammatory process in the skin. Quitting smoking is therefore essential. The whole body must be warm at all times, because the cold induces signal substances in the body that constrict the blood vessels. Patients must therefore dress very warmly (all over their bodies), stay in well-heated rooms, wear heated gloves in winter and use pocket warmers. A winter vacation in the south is also recommended. To prevent constipation, patients need to consume lots of lettuce, fruit, whole grain bread and similar fiber, supplemented with plenty of fluids. Dietary advice is recommended in individual cases. In order to prevent the skin and mucous membranes from drying out, sufficient fluid intake and regular skin care are necessary, and dust and wind (i.e. especially long car trips, road work, construction work) are to be avoided. Injuries to the hands must be prevented by means of suitable industrial safety measures. Continuous physiotherapy (e.g. lymph drainage and physiotherapy) is essential for maintaining joint mobility. Appropriate, regular physical activity (e.g. swimming in warm water) helps relieve stress and anxiety, maintain joint and muscle functions, expand blood vessels and exercise the heart. In the cold season, more preventive measures and more active therapy are necessary.
Further information can be found on the websites of other institutions under Links. If you have any questions about your illness, please contact us personally during our consultation hour. An information day for scleroderma patients takes place every year at our clinic.
According to the current state of knowledge, systemic scleroderma is not permanently curable, but only treatable. In this respect, regular medical checks are essential. The team of our autoimmune consultation is at your disposal.
Before you introduce yourself in our special consultation, we ask you to fill out our anamnesis sheet.
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