JUVENILE DERMATOMYOSITIS (JDMS)The Arthritis Foundation
with appreciation Suzanne L. Bowyer MD,
JUVENILE DERMATOMYOSITIS (JDMS) Back to padeh.net
The Arthritis Foundation acknowledges
with appreciation Suzanne L. Bowyer MD,
Pediatric rheumatologist at the Children's
Pediatric rheumatologist at the Children's
Hospital of Los Angeles, Los Angeles, California and Charles H. Spencer, MD, pediatric rheumatologist, at the National
Institutes of Health, Bethesda, Maryland,
for their assistance in the preparation of
@ 1988 by the Arthritis Foundation
This booklet is about juvenile dermatomyositis (JDMS), a disease that affects 3,000 - 5,000 children in the United States. It is written for parents or other adults who know a child who has JDMS, or for young adults who have JDMS. This booklet is not meant to take the place of treatment and teaching provided by a doctor and other health professionals, but it should help you understand JDMS in your child. The Arthritis Foundation has other booklets about rheumatic diseases * in children-Contact your local chapter for more details.
JDMS affects every child differently. The disease can be mild in some children and serious in others. This booklet describes the general symptoms of and treatment for JDMS, but it is important for you to discuss your child’s particular case with your doctor. If you have questions as you read the booklet, write them down and take the written list to your doctor.
Juvenile dermatomyositis (der-MA-toe-MY-o-SY-tis) (}JDMS) is a disease that causes a skin rash and weak muscles in children. }JDMS is different from dermatomyositis in adults, and from polymyositis * (PAH-lee-MY-o-SY-tis), a disease that usually affects adults.
The skin rash and weak muscles of JDMS are caused by inflammation in the blood vessels that lie under the skin and in the muscles. This is called vasculitis. Since blood vessels run throughout the body, JDMS can also affect other areas such as the digestive tract. Usually, the worst symptoms are in the skin and muscles. JDMS is often called an autoimmune disease. The immune system is a group of cells which normally protects the body from infections. In autoimmune diseases, these cells are signaled to “turn on” their infection-fighting process, but then can’t turn it off. The process then ends up damaging the body rather than protecting it. One way the immune system cells fight infection is by a process called inflammation (in-flah-MAY-shun). But when the cells can’t “turn off” the inflammation process, tissues are damaged. In JDMS, blood vessels in the skin and muscles are inflamed. An inflamed muscle will feel weak or sore, while inflamed skin will have a rash.
We don’t yet know what causes JDMS and we don’t know what causes the immune system to damage the body. Many researchers now think the autoimmune reaction may be caused by the immune system’s abnormal response to a virus. That is, immune system cells attack an invading virus, but then also attack the body’s own tissue. We now think the tendency for the immune system to react in this way may be at least partially inherited.
JDMS is not contagious. Your child didn’t catch it from another child, and nothing you could have done would have prevented your child from developing this disease.
There are three main symptoms of JDMS: weak muscles, skin rash, and fever.
The skin rash and weak muscles are usually the first signs of the illness. Some children may also have other symptoms such as sore joints or less-common symptoms that occur later in the illness.
The muscles in and near the trunk, such as the neck, shoulders, back, abdomen, and hips will slowly become very weak. The muscles used for swallowing and breathing may also become weak.
What you may notice: Your child is likely to have trouble with common movements such as c1imbing stairs, riding a bicycle, or getting out of a bathtub. It may also be difficult to get up from the floor or from a low chair. Your child will probably have a hard time keeping up physically with children the same age, may fall more often than usual, and may often feel tired. Children with JDMS may also complain that their muscles are sore to touch.
In very severe cases of JDMS, even the muscles used for swallowing can be affected. Your child may have trouble swallowing chunks of food such as meat, and may choke on some foods. These same muscles are used for speaking, so your child's voice may become weak, and she may sound as if she is talking through her nose. If you or your child notice any of these symptoms, contact the doctor immediately
Major muscle groups most often affected By JDMS
The skin rash usually occurs on the face; knuckles; elbows; knees; and ankles. In some children; the rash may cover the whole body. The rash may appear before; after; or at the same time as the muscle weakness. The rash may be made worse by sunlight.
What you may notice: Your child’s eyelids and cheeks may appear red or purplish, and the eyelids may become puffy. Red patches that look like dry skin often appear over the knuckles, elbows, and knees. The skin around the fingernails may appear red.
Because of the puffy eyelids and skin rash, this part of the illness can look very much like an allergy. Since the rash may be made worse by sunlight, children should wear sunscreen when they are in the sun-
At the start of JDMS, some children have a fever along with the other symptoms described above.
What you may notice: Your child’s temperature will rise one or two degrees.
The following symptoms may also occur in JDMS. Some are less common, but since they can occur you should know about them.
In JDMS, one or several joints may become stiff or inflamed (swollen). The joints feel stiff or sore because the muscles around them are inflamed, or because the joint itself is inflamed. Unlike other forms of childhood arthritis, the inflammation usually doesn’t last long or cause severe joint damage.
What you may notice: Your child may complain of stiff and sore joints. The joint may also feel a bit warm when you touch it.
Calcinosis (kal-sih-NO-sis) is a process that causes small lumps of calcium to form under the skin or in the muscle. The lumps may feel like little rocks under the skin and can range from the size of a period on this page to the size of a flat pebble. Sometimes, the smaller lumps join together to form larger calcium lumps. When this happens, it can impair muscle movement. Too much calcium in your child’s diet does not cause the lumps - they are a late result of the illness and occur in more than half of all children with JDMS.
If the calcium lumps are bumped a lot, they can become sore. This is most likely to happen if they are on your child’s knee, elbow, buttocks, or other pressure point. Small pieces of creamy white calcium may break through the skin and drain from the hole. These draining areas will usually heal, but they can become infected. Con-tact your doctor if this happens. After a while, the calcium lumps are usually absorbed back into the body.
A vasculitic ulcer is a hole in the tissue that surrounds an inflamed blood vessel. In JDMS, vasculitic ulcers can occur in the skin and in the bowels (the pathway of organs through which food travels from the esophagus to the rectum).
In the skin. These ulcers happen when the blood vessels under the skin are severely inflamed. They look like sores in the skin rash, and can be as small as a pen-ell point or larger than a silver dollar. Some ulcers are very deep, while others occur only on the surface- they can be very painful, and can last anywhere from a few days to several months- the ulcers usually go away with treatment.
In the bowels: These ulcers happen when the blood vessels in the protective lining around the bowels are inflamed. This causes a break, or hole, in the lining. Strong digestive juices can then leak through that hole and damage digestive organs. If this happens to your child, you may notice these symptoms:
· . severe pain in the abdomen
· . coal-black stools
· . change in pattern of bowel movement
· . passing blood in stools
This happens only rarely, but if you or your child notice any of these symptoms contact the doctor immediately.
A contracture is a shortened muscle that causes a joint to remain in a bent position. In JDMS, there are two ways a contracture may occur:
Through the healing process: As the muscle heals, it may scar. These scars can shorten the muscle, and pull the joint into a bent position.
Through a lack of exercise: At times, your child’s muscles might feel too sore to exercise. Without exercise, the muscles become weak. Because a weak muscle cannot hold the joint in its correct, extended position, a contracture will occur. You and your child can help prevent serious joint contractures with daily exercises and proper physical therapy (see p. 15).
When the body is fighting a disease, it uses a lot of the energy, which it would normally use for growth. Because your child may not feel well, she may not feel like eating. These factors, along with some of the medicines used in treatment, may cause your child to grow and mature more slowly than normally. Usually, your child will begin to grow again once the disease is under control.
In some children, the signs of JDMS develop over a long period of time. In others, the signs develop quickly. To find out if your child has JDMS, the doctor will ask about your child’s health history, perform a complete physical examination, and obtain lab-oratory tests.
The doctor will ask many questions about your child’s symptoms, such as:
· When did the symptoms start?
· What parts of the body are involved?
· Are the symptoms always present, or present only at certain times?
· Are the symptoms getting better or worse?
The doctor may also want to know if other members of the family have had arthritis or a muscle disease since, in rare instances, some forms of these illnesses may run in families.
The doctor will examine your child, paying special attention to skin and muscles. In particular, the doctor will look for these signs:
knees, and/or fingertips
· swelling around the eyes
· nasal speech pattern
· sore and/ or weak muscles
· sore and/or swollen joints
The doctor will also check your child’s strength and will check routine areas, such as the eyes, ears, nose, and throat.
Laboratory tests help determine if your child has JDMS. These tests show if there is inflammation in the muscles or in other areas of the body. Many of these tests may be abnormal early in the course of the illness, even if your child has only mild muscle weakness. The most common tests are:
When the muscles are inflamed or dam-aged, certain muscle proteins (called enzymes) leak into the blood stream. The doctor will look for enzymes such as CPK and aldolase. If large amounts of these enzymes are found in the blood, then the doctor knows that some muscles have been damaged.
Antinuclear Antibodies (ANA)
This test shows whether or not your child’s immune system is producing anti-bodies against the body’s cells.
This test measures electrical activity in the muscles. In this test, a thin needle is placed through the skin and into the muscle. A wire runs from the needle to a machine that records electrical activity in the muscle. Children with JDMS have a specific electrical pattern that shows the muscle is inflamed.
This test shows how much the muscles or blood vessels have been affected by the disease. If your child needs a biopsy, the doctor will remove a small piece of muscle and look at it and its surrounding blood vessels under a microscope to see if there is any inflammation. Children with damaged blood vessels tend to have more problems during their illness.
By putting together the story of how the illness started with the results of the physical examination and the laboratory tests, the doctor can usually make the proper diagnosis.
There is no cure for JDMS, but treatment can help prevent or control most serious problems. Your child’s treatment plan will be based on the severity of the illness. The goals of any treatment program for JDMS are to :
· control muscle inflammation and damage
· maintain and improve muscle strength and function
· relieve pain
· control or prevent other symptoms
· help child and family learn to live with the illness
To reach these goals, treatment usually includes:
· a balanced diet
As your child’s symptoms change, the treatment plan may also be changed.
There are several medications your doctor may use to treat JDMS. The specific drug or combination of drugs your doctor uses depends on the type and severity of your child’s symptoms. Since only common side effects of each drug are listed here, you should discuss your child’s medications with the doctor.
Prednisone slows down the immune system and helps control inflammation in the muscles and joints. This drug is similar to cortisone, a natural body hormone.
Prednisone is used to:
· relieve muscle and joint pain
· improve muscle strength
· control fever
· Control skin rash Dose: At first, high doses of this drug are given until your child’s muscle enzyme tests improve. As your child gets better, the dose will gradually be lowered.
Common side effects: The side effects your child may have depend on how much medicine the child takes, and how long she has taken it. Common side effects include :
· increased appetite
· weight gain
· stretch marks on the skin
· round, full cheeks
· high blood pressure
· mood changes
Over a long period of time, the drug can also cause these problems:
· weak or damaged bones
· small cataracts
· slowed growth rate
· greater chance of developing diabetes, if it runs in the family
Prednisone may cause other side effects, depending on the dose and length of time your child takes it. Discuss the side effects with your child’s doctor. This drug is given because the benefits of controlling the JDMS outweigh the risks of side effects. The doctor will try to lower the dose as soon as possible to lessen the side effects, while keeping the disease under control. A word about relapses: as the prednisone dose is lowered, your child’s earlier symptoms of JDMS may return. This is called a relapse. These symptoms will usually go away with a mild increase of prednisone. Although some children may have more than one relapse, a relapse may not necessarily slow your child’s recovery from JDMS.
If prednisone alone can’t control your child’s illness, a stronger drug such as methotrexate or cyclophosphamide (Cytoxan) is often used. These drugs also slow down the body’s immune system. Dose: The doctor will use as little of these drugs as possible and will usually use them in combination with prednisone. The drugs are given as a pill or by injection. As your child gets better, the doctor will slowly reduce the dose of one or both of the drugs. Common side effects: Because these drugs can cause serious side effects, they are used only if other medicines have not controlled the disease well enough. Talk to your doctor to decide whether the benefits of the drug outweigh the risk of its side effects. Side effects may include:
· nausea and vomiting
· mouth sores
· skin rash
· damage to some internal organs
· decreased number of blood cells
These side effects will go away when the dose is lowered or stopped completely. These drugs may cause other, more serious side effects. If your child is taking any of these drugs, the doctor will take regular blood and urine tests to monitor the drug’s effect on your child.
Hydroxychloroquine (hi-DROCKS-ee-CLOR-o-kwine) is a drug sometimes used to treat a severe rash in JDMS. The brand name of this drug is Plaquenil (PLA_kweh-nill).
Dose: This drug is given as a pill. Since it
simply controls the rash, your child will
will also take other medicines for other symptoms.
Common side effects: Side effects include:
· upset stomach
· eye problems
A small dose of Plaquenil is given for JDMS, so eye problems don’t happen very often. Plaquenil may cause problems such as trouble focusing sharply on an object, or seeing a “halo” around lights. In the earliest stages of eye damage, the damage is still reversible. At this early stage, your child may not notice any symptoms. That is why an ophthalmologist (eye doctor who is an MD) should check your child’s eyes every three to six months while your child is on Plaquenil. If eye problems are detected early, they should go away when the medicine is stopped.
Exercise is a very important part of JDMS treatment: in fact, it is nearly as important as medication. The purpose of regular exercise is to:
· keep joints flexible
A physical therapist will teach your child exercises to do at home. These exercises will be designed specifically for your child and will change depending on the activity of the disease. At first, your child’s muscles may be weak and she may need help with the exercises. Later on, when your child won’t need as much help, the exercises will be changed.
Exercises usually take about one hour per day. It is important for your child to do the exercises regularly. But not to overwork and tire her muscles. The therapist will try to combine some exercises with your child’s normal play activity.
Although there is no diet that will cure JDMS, it is important for your child to eat a regular, balanced diet. Protein in your child’s diet is important for normal growth and to repair damaged muscles. Calcium is important for strong bones. If your child does not feel well and does not want to eat, try to keep her interested in foods by letting her help prepare meals, and by preparing many different foods. A nutritionist can help plan a good diet for your child.
Your doctor, nurse and other health care workers can help teach you about JDMS. By teaming about the illness and how to treat it, both you and your child will be able to take better care of the disease.
Your child’s regular doctor is the best person to see when your child first becomes ill.
Because JDMS may be hard to diagnose or treat, the doctor may send your child to a specialist such as a pediatric rheumatologist, a doctor who specializes in childhood arthritis. If there is no pediatric rheumatologist in your area, your doctor may refer you to another specialist who is knowledgeable about JDMS.
Several other health professionals may be involved in your child’s treatment program:
· Nurse - teaches your child about JDMS and how to take care of it. This includes teaching pain control and play therapy to help the child deal with the illness.
· Physical therapist - tests muscle strength and designs exercises which strengthen large muscles I such as the arms and legs) and keep joints flexible. A physical therapist can also help your child regain lost coordination and movement.
· Occupational therapist - teaches new ways to do daily activities such as eating, combing hair, and dressing, which may be more difficult for a child with JDMS. These activities strengthen smaller muscles (such as those in the hand) and help the child feel more independent.
· Social worker – helps with the personal, emotional, family, or financial problems that may have occur with JDMS.
Other persons such as nutritionist, pharmacist, psychiatrist or psychologist can help you handle special problems.
Try not to overprotect your child, even though this is a natural desire. Your child might become too dependent if you do everything for her or if you do tasks, which she is capable of doing.
Don’t be “blackmailed” into allowing activities that shouldn’t be done, but com-promise when you can. Being as consistent as possible will help your child team what is expected.
Plan special time to spend alone with your spouse, or with the entire family. When your child first becomes ill, you may set aside relationships with other family members. It is important, however, to continue to talk to and spend time with all family members.
Overall, it is important for the whole family to fit JDMS into its regular life pat-tern and to adapt to the illness rather than fight it. This means fitting your child’s treatment program into the family’s regular daily schedule. It means asking for special help from people when you need it. Adapting also means talking (and crying) about JDMS now and then. Of course, JDMS will make life more difficult than it used to be - but accept this as a challenge rather than a burden.
WHERE CAN YOU GO FOR MORE HELP?
Sometimes, the stress of having JDMS may cause your child to develop emotional or behavioral problems that you cannot deal with alone. Other family members may also feel overwhelmed. Whatever the case may be, counseling or support groups can help work things out. Sometimes all that is needed is a talk with a sympathetic person, another family who has a child with a rheumatic disease, or a professional who is trained to help in these situations. The doctor, nurse, or medical social worker may be able to help you or refer you to other sources of help.
Generally, children with JDMS should attend a regular school and should not be isolated from other children their same age. But because of JDMS your child may need special services, such as special transportation or supplies, to help her get along in a regular school.
How can you help your child avoid problems at school?
· Be informed
· Inform others
There are two federal laws, which affect your child’s rights in school:
Section 504 of The Rehabilitation Act of 1973
This law forbids all federal programs from discriminating against disabled persons. It means your child should be able to receive services from all programs and agencies (including schools) that receive money from the federal government.
Public Law 94-142 (The Education for All Handicapped Children Act)
This law says that every child has a right to a free public education, whether or not the child has a disability. It means you can work with your child’s school to make sure she receives the services she needs in order to attend public school.
For more details about these laws see When Yom Student Has Arthritis, available from your local Arthritis Foundation chapter.
If you have problems protecting your child’s rights, you can contact the following organizations:
The American Juvenile Arthritis Organization
1314 Spring Street, N.
Atlanta, Georgia 30309
The Arthritis Foundation’s national membership organization that focuses on the problems related to arthritis and rheumatic diseases in children.
The Federation for Children With Special Needs
312 Stuart Street
Boston, Massachusetts 021 16
A national organization that helps children with special needs obtain an equal opportunity for education.
Muscular Dystrophy Association
810 Seventh Avenue
New York, NY 10019
A national organization that provides support services for persons with neuromuscular disorders.
National Information Center for Children and Youth with
A national resource center for parents of disabled children.
Talk to people at the school about your child’s illness. Tell your child’s teachers, the school nurse, and the principal about:
· how JDMS affects your child, and problems that may occur in school
· your child’s treatment program, including medications taken during school hours
· how to reach you or your child’s doctor, if necessary
· this booklet and other Arthritis Foundation booklets such as, When Your Student Has Arthritis
Most children have active JDMS for about two years. Since JDMS affects every child differently, some children may have active disease longer than two years, and some may have more severe symptoms than others.
JDMS is a treatable disease. Although most children recover completely from the disease, some children will have permanent muscle weakness from it. Despite the challenges children with JDMS and their families face, the majority of these children grow up to lead a fulfilling, happy life.
By reading this booklet, you have begun to learn about your child’s condition and how to treat it. Most importantly, you have learned some of the ways to approach the daily challenges of the illness. Continue to read and learn about JDMS. You will be helping yourself, your child, and your family adjusts to the challenges of the illness: you will be helping them to an active and happy future.
Antinuclear antibody (ANA) - a type of protein found in the blood of some children with different forms of autoimmune disease.
Arthritis - general term meaning inflammation of a joint.
Connective tissue - tissue that supports, binds, and/or separates other tissues and organs. Bones, cartilage, and tendons are types of connective tissue.
Chronic disease - an illness which lasts for a long time, (months to years) or for the life of the affected individual.
Juvenile rheumatoid arthritis - a form of arthritis that occurs in children. Its most characteristic feature is joint swelling and pain.
Polymyositis - (PAH-lee-my-o-SI-tis) a rheumatic illness that affects muscles. It occurs most frequently in adults. Polymyo-sitis is different in many ways from JDMS and from dermatomyositis in adults.
Rheumatic disease - (roo-MA-tick) general term for diseases that involve inflammation of connective tissue. Juvenile dermatomyositis, juvenile rheumatoid arthritis, scleroderma, and systemic lupus erythematosus, are types of rheumatic diseases that affect children.
Systemic lupus erythematosus- (ee-RIH-them-a-TOE-sus) a chronic inflammatory disease which can affect skin, muscles, joints, and internal organs.
The mission of the Arthritis Foundation is to support research to find the cure for and prevention of arthritis and to improve the quality of life for those affected by arthritis.
Formed in 1948, the Arthritis Foundation is the only national, voluntary health organization that works on behalf of all people with any of the more than 100 forms of arthritis or related diseases. Volunteers in chapters nationwide help to support research, professional and community education programs, services for people with arthritis, government advocacy and fund-raising activities.
In 1981, the Arthritis Foundation established the American Juvenile Arthritis Organization (AJAO) - a national member-ship organization, which works as a part of the Foundation to focus its efforts on the specific problems, related to arthritis in children.
This group was formed for children with arthritis and rheumatic diseases, for their parents and friends, and for health professionals who treat these children. The AJAO publishes a newsletter and provides information about arthritis in children to parents and the general public.
Some chapters of the Arthritis Foundation have helped parents organize AJAO groups. Many of these groups meet regularly and provide informative programs on the medical, financial, and emotional issues surrounding arthritis in children. The groups also provide an opportunity to talk with other parents of children with arthritis. Contact your local chapter of the Arthritis Foundation for more information about AJAO and local parent groups.