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Forms of Scleroderma
There are two major classifications of scleroderma: localized scleroderma and systemic sclerosis (SSc). Other forms or sub classifications, each with its own characteristics and prognosis, may be identified through future research.
Localized Scleroderma The changes, which occur in localized scleroderma, are usually found in only a few places on the skin or muscles, and rarely spread elsewhere. Generally, localized scleroderma is relatively mild. The internal organs are usually not affected, and persons with localized scleroderma rarely develop systemic scleroderma. Some laboratory abnormalities commonly seen in systemic scleroderma are frequently absent in the localized form.
- Morphea is a form of localized scleroderma characterized by waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken. The patches may enlarge or shrink, and often may disappear spontaneously. Morphea usually appears between the ages of 20 and 50, but is often seen in young children.
- Linear scleroderma is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound. Linear scleroderma tends to involve deeper layers of the skin as well as the surface layers, and sometimes affects the motion of the joints, which lie underneath. Linear scleroderma usually develops in childhood. In children, the growth of involved limbs may be affected.
Here are some useful definitions of medical words and terms from NSF.
Acid reflux, heartburn. Stomach acid, which abnormally travels up into and irritates the esophagus. (Acid production is a normal part of digestion in the stomach.) Heartburn refers to pain in the center of the chest caused by acid reflux. (See Esophagitis.)
Analgesic. A medication, which reduces or eliminates pain. Example: aspirin and non-steroidal anti-inflammatory drugs.
Antacid. An agent, which neutralizes excess stomach acid. This may be liquid/tablets, which act immediately in the stomach, or long-acting medications taken regularly and absorbed into the blood in order to suppress acid production. (See Acid reflux.)
Antibiotic. Medication used to treat an infection. Each antibiotic kills or inhibits the growth of specific microorganisms, so antibiotics are prescribed based on the type of infection present.
Arthralgia. Pain in a joint.
Autoimmune. Disease or antibody, which acts against the person’s own tissues. (See Immune system.)
Biofeedback. A technique used to regulate a body function usually involuntarily controlled, such as a finger temperature or pulse rate. By observing a machine monitoring the function, a person can practice relaxation techniques and learn to control the function. Later, the machine becomes unnecessary. (See Relaxation techniques.)
Biopsy. The removal and examination of tissue, cells or fluid from the body.
Blanched. To become white or pale. In Raynaud Phenomenon, the fingers and toes blanch due to insufficient circulation of blood.
Calcinosis. Abnormal accumulation of calcium in the skin.
Capillaries. The smallest blood vessels of the body, connecting arteries and veins.
Collagen. A normal, fibrous protein found in the connective tissue of the body.
Connective tissue. Tissue, which pervades, supports and binds together other tissues including mucous, fibrous, reticular, adipose, cartilage, skin and bone. Connective-tissue diseases are a group of diseases with similar cellular changes, but with the site where the changes occur determining the specific disease. Included are scleroderma, systemic lupus erythematosus, dermatomyositis and rheumatoid arthritis.
Constrict (vessels), stricture (esophagus). An abnormal narrowing.
Contraction (of intestinal muscles). The rhythmic squeezing action of the muscles of the wall of the intestine, which moves food through the system. Also called peristalsis. (See Motility.)
Coronary arteries. Blood vessels, which supply blood to the heart itself.
CREST. Form of scleroderma, whose initials stand for Calcinosis, Raynaud Phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia.
Cutaneous. Of the skin.
Cyanosis. Blue or purple color due to lack of blood oxygen. In Raynaud Phenomenon, cyanosis of the fingers and toes may follow blanching.
Digits. Fingers and toes.
Dilate (esophagus, blood vessels). To widen or enlarge.
Diuretic. Medication to increase the flow of urine, thereby decreasing fluid retention in the tissues. Also called “water pills.” (See Edema.)
Dysfunction, disfunction. Impaired or abnormal functioning.
Dysphagia. Difficulty in swallowing.
Edema. An abnormal excess accumulation of fluid in tissues or cavities of the body.
En coup de sabre. A form of localized scleroderma which forms a long crease of waxy skin, resembling a cut by a saber or sword wound usually on face or neck.
Esophagus, esophagitis. The muscular swallowing tube connecting the mouth and the stomach. When properly functioning, it contracts in smooth waves to send food to the stomach. At its lower end a sphincter (ring-like muscle) opens to allow food to pass into the stomach but closes again to prevent stomach acid or partially digested food from backing up into the esophagus. Esophagitis is an inflammation or irritation of the esophagus.
Fatigue. Weariness, a sense of being overwhelmingly tired, or exhaustion.
Fibrous. Consisting of or resembling fibers.
Fibrosis. Abnormal formation of excess fibrous tissue.
Gastrointestinal tract, bowel, diarrhea, constipation. The gastrointestinal tract is the digestive system, which breaks down food, allows absorption of nutrients, removal of cellular waste products, and elimination of solid waste from the body. It begins with the mouth and esophagus and leads to the stomach. The small intestine consists of the duodenum, jejunum and ileum. Lastly, the large intestine (also called colon) leads to the rectum. The term bowel refers to the intestine. The anal sphincter is the muscle, which controls discharge of stool. Diarrhea is abnormally frequent or excessive passing of stool, usually watery. Constipation is the abnormally delayed or infrequent passage of stool, usually in a dry and hardened state. Normal bowel movements vary from person to person and with diet.
Hypertension, anti-hypertensive. Abnormally high blood pressure. An anti-hypertensive medication lowers blood pressure.
Immune system. The system of organs, cells, and proteins, which protect the body from foreign substances by producing immune responses. The immune system organs include the thymus, spleen, lymph nodes and bone marrow. The cells include white cells, lymphocytes, T cells and B cells. Immunoglobulins (antibodies) are proteins that can react with and/or neutralize corresponding proteins called antigens (usually damaged or foreign material). The immune system is essentially protective and helpful to the body, but can be the cause of disease and allergy when it attacks parts of the normal body in a process called autoimmunity.
Inflammation, anti-inflammatory. Tissue reaction to cell injury marked by redness, heat, pain, swelling and often loss of function. Capillary dilation and white blood cell infiltration help eliminate foreign substances and damaged tissue, so normally; inflammation is a natural part of the healing process. Excessive or inappropriate inflammation can, however, cause further damage. Anti-inflammatory drugs counteract inflammation.
Joint contracture, flexion contracture. Fixation of a joint in one position preventing full range of motion. In scleroderma, frequently affecting the fingers, due to tightening and hardening of the skin around the joint. In flexion contractures, the fingers become fixed in a bent or flexed position.
Juvenile Localized Scleroderma (jLS, also referred to as Pediatric Localized Scleroderma)
Juvenile Systemic Sclerosis (jSSc, also known as Pediatric SSc)
Lacrimal glands. Tear-producing glands, also spelled lachrymal.
Laxative. A medication, which stimulates emptying of the bowels.
Lubrication, secretion. Substance which makes a surface slippery or oily, either artificially by applying lubricating fluids or naturally by secreting fluids made by cells for this purpose. Example: tears.
Malabsorption. The reduced ability to take nutrients from food into the cells of the body from the digestive tract.
Microstomia. Abnormally small mouth opening.
Mixed Connective Tissue Disease. Overlap or presence of symptoms of two or more diseases simultaneously. (See Collagen and Connective tissue.)
Morphea. A form of localized scleroderma.
Motility, dysmotility. Contractions of the digestive-tract muscles occurring in rhythmic waves, propelling food, allowing absorption of nutrients, and elimination of wastes (feces). Dysmotility indicates weakened or absent waves of contraction resulting in abnormally slow movement of food and feces. (See Malabsorption, Gastrointestinal tract, Contraction.)
Occupational therapy. Therapy using activity prescribed to promote recovery or rehabilitation. Often designed to increase ability to perform acts of daily living, such as grooming and eating, and concentrating on the hands and small muscle control.
(Abbreviated “OT.” See also Physical therapy.)
Ophthalmic. Related to, or situated near the eye.
Pediatric Localized Scleroderma (also referred to as Juvenile Localized Scleroderma, jLS)
Pediatric Systemic Sclerosis (also known as Juvenile Systemic Sclerosis, jSSc)
Pericarditis. Tissue inflammation of the sac enclosing the heart.
Peripheral blood circulation. The flow of blood to the arms and legs.
Phenomenon. An unusual, significant, or unaccountable fact or occurrence which, when observed, is of scientific interest.
Physical therapy. Treatment of disease and injury by mechanical means such as massage, regulated exercise, water, light, heat and electricity. Often concerned primarily with joint motion, large muscle groups, and activities such as walking and aerobic and isometric exercise. (Abbreviated “PT.” See also Occupational therapy.)
Pleurisy. Tissue inflammation of the sac enclosing the lungs.
Prognosis. Prediction of the progression and end result of a disease, or estimate of chance of recovery.
Pulmonary fibrosis. A process in which the lungs are scarred, decreasing the transfer of oxygen to the blood. Also called restrictive lung disease.
Pulmonary hypertension. Elevated pressure in the blood vessels of the lungs, decreasing blood oxygen and straining the right side of the heart.
Raynaud Phenomenon. Also called Raynaud Syndrome. A disorder with recurring spasms of the small blood vessels upon exposure to cold; characterized by fingers and toes turning white, blue, and red as circulation abnormally overreacts to normal conditions. Emotional stress may also trigger an attack. Named for the French physician (Dr. Maurice Raynaud, pronounced “Raynode”) who first described it.
Relaxation techniques. Stress-reducing procedures, which can also be used to help regulate body functions such as finger temperature or pulse rate. These include tensing and relaxing muscles, imagery, breathing techniques, and medication. (See also Biofeedback.)
Remission, spontaneous remission. A period during which the symptoms of a disease decrease or go away. If the reason for remission is not related to treatment but seems to occur for no apparent reason, it is called spontaneous.
Renal. Relating to the kidneys.
Respiratory. Pertaining to breathing or the lungs.
Salivary glands. Glands, which secrete fluid (saliva) into the mouth.
Sclerodactyly. Thick, tight skin of the fingers and/or toes. (See Joint contracture.)
Sclerosis. An abnormal hardening of tissue.
Sjögren’s. A chronic inflammatory disease characterized by decreased secretions, especially dry eyes and dry mouth, named for the Swedish physician who first described it. It may occur alone, or as a part of scleroderma or other autoimmune diseases. (Pronounced “show-grenz.”)
Skin ulceration. A break in the skin with loss of surface tissue. It may also be associated with inflammation, calcium deposits and infection.
Spasm. Involuntary and abnormal contraction of muscle.
Stasis. A slowing or stoppage of body fluids as in venous stasis. Also, reduced motility of the intestines with retention of feces.
Systemic. Affecting the whole body rather than one of its parts. Opposite of localized.
Telangiectasia. An abnormal dilation of skin capillaries causing red spots on the skin.
Vascular. Pertaining to or composed of blood vessels.
Vasodilator. A medication (or other substance) which causes widening of blood vessels.
