What Are the Long-term Sequelae of Dermatomyositis?

Patient Presentation
A 10-year-old female was admitted to the hospital for an evaluation of possible dermatomyositis.
She first had symptoms 6 months prior to admission when she developed a scaly, red rash on her hands, elbows, knees, bilateral cheeks and eyelids.
Shortly after this rash appeared she began having stiffness in her hands. She had no fevers, weight loss or other systemic problems.
She was seen by an adult dermatologist who diagnosed psoriasis. Since that time, despite dermatological treatment, she has not had improvement.
She needed assistance with activities of daily living at home and at school because of pain and generalized weakness felt to be secondary to deconditioning.
She was referred to a pediatric rheumatologist who believed the differential diagnosis included psoriatic arthritis, dermatomyositis or an overlapping syndrome with another similar disease process.
She was begun on prednisone with improvement of the arthritis, however 4 weeks later, her rash was now progressing on her arms, legs and feet and was violaceous around her eyes and upper cheeks.
She also complained of increasing pain in her right elbow and had difficulty moving it. She said that she also felt weaker.

The family history is negative for joint, muscular, neurological, kidney or small vessel abnormalities.
There is a maternal aunt with migraine headaches, another maternal aunt with psoriasis and other family members have eczema.
Some family members are allergic to penicillin.

The review of systems is positive for 3 days of abdominal pain that was diffuse and intermittent. She had not had a bowel movement for 3 days and had pebbly stools at baseline.
She denied blood per rectum. She also has occasional nausea, but no vomiting or acid reflux in her mouth. She has no fever or urinary complaints.
The pertinent physical exam showed a cooperative girl who had mild pain in her right elbow and no abdominal pain.
She had growth parameters at ~75%. She as afebrile and had a blood pressure of 115/68.
She had mild bilateral yellow-white discharge on her eyelids with mild conjunctival injection. Her lips were dry with mild cracking without bleeding.
Lungs are clear. Cardiac examination is normal with no rubs or murmurs.
Abdomen was slightly distended but soft with normal bowel sounds. She had stool palpable throughout the colon. She had mild tenderness with deep palpation and no rebound. Stool guaiac was negative.
There was no organomegaly.
Skin examination showed a violaceous rash around the eyes and cheeks with mild edema. She also had erythematous, maculopapular patches with mild scaling on the face, arms, legs, chest, abdomen, back and inguinal area. She had golden yellow crusting on some areas of her lower legs.
Extremity examination showed her right elbow was contracted, red and warm. Additionally, she had full flexion but limited extension of the elbow. She had swollen fingers.
Neurological evaluation showed cranial nerves and deep tendon reflexes to be normal. She had decreased strength in her neck (4/5), trunk (3/5) and extremities (3/5). She had a normal gait but needed assistance secondary to her generalized weakness.
An extensive laboratory evaluation was done including a complete blood count showing mild anemia, and increased aldolase, transaminases, lactate dehydrogenase and creatine phosphokinase.
She had a normal C-reactive protein. All other testing was negative
The radiologic evaluation included an right elbow radiograph which was normal and a right elbow magnetic resonance that showed mild subcutaneous edema but no effusion.
Abdominal radiograph showed stool present throughout the colon.
She also had a magnetic resonance imaging of her pelvis which showed findings within the pelvic musculature consistent with dermatomyositis.
After the pelvic imaging the rheumatologists felt that diagnosis of dermatomyositis was probable and felt that a muscle biopsy or electromyographic abnormalities was not needed to further confirm the diagnosis.
The patient’s clinical course was that she was begun on pulse steroid therapy with Solu-Medrol for 3 days by IV infusion for 3 days. Etanercept to be given weekly. She was not started on methotrexate because of her elevated liver function tests.
She was evaluated by physical and occupational therapy who prescribed range of motion exercises and some accommodations for activities of daily living.
Her abdominal pain improved after treatment with Miralax® and subsequent bowel movement. the Miralax was continued as prophylaxis for her constipation.
She was also given lansoprazole and ranitidine for ulcer prophylaxis secondary to her steroids.
for her impetigo she was treated with clindamycin IV and then changed to oral medication with resolution.
Ophthalmology consult confirmed conjunctivitis and recommended gentamicin ophthalmic drops because of her immunosuppression and risk of infection. A more comprehensive ophthalmologic evaluation was recommended as an outpatient.
Her anemia was felt to be secondary to chronic disease.
Her blood pressure remained normal during admission and did not require treatment.
At follow-up 10 days later, her rash is slowly resolving and she is having some increased range of motion in her elbow. She continued to be weak however. Her liver function tests were returning to normal and she was to begin methotrexate soon.
Other laboratory testing for disease monitoring was being completed.

Figure 57 – Axial T2-weighted (above) and post-contrast T1-weighted (below) MRI images through the level of the hips demonstrate small patchy areas of increased T2 signal and mild enhancement bilaterally and symmetrically in the gluteus muscles and in the subcutaneous tissues of the lateral and posterior thighs. These findings were felt to be compatible with mild dermatomyositis.

Juvenile dermatomyositis (JDM) is a disease causing inflammation of small vessels in multiple organs. Its etiology is unknown, but possibly is autoimmune in origin. Overall incidence is 2-3/million with females more affected than males.
Over the past 40 years, there has been a marked improvement in survival (mortality is < 3%) and functionality.

In one prospective study of JDM patients presenting symptoms, they had: rash (100%, i.e. heliotrope, Groton papules or malar/facial rashes often), weakness (100%), muscle pain (73%), fever (65%), dysphagia (44%), hoarseness (43%), abdominal pain (37%) and arthritis (35%).
Another study found that JDM patients presenting symptoms had: rash (42-91%), fever (16%), dysphonia (24%), pulmonary problems (11%), arthritis (6%), and gastrointestinal problems (5%).

Learning Point
Patients with JDM can have many long-term sequelae.

  • Calcinosis – seen in 22-40% of patients – usually in sites of trauma late in the disease.
  • Gastrointestinal abnormalities – seen in 22-37% of patients – vasculopathy may cause hemorrhage, perforation or ulceration. Esophageal dysmotility, malabsorption and pneumatosis intestinalis have occurred.
  • Growth problems – seen in approximately 30% of patients – these patients were at least 1 standard deviation below their predicted height.
  • Lipodystrophy and metabolic problems – seen in 14-25% of patients – slow, progressive symmetrical loss of subcutaneous fatty tissue that may be generalized, partial or localized. There may be associated metabolic problems including
    acanthosis nigricans, clitoral enlargement, hirsutism, hepatomegaly, insulin resistance, menstrual abnormalities, and hypertriglyceridemia.

  • Nailfold capillary changes – seen in 80-100% of patients – The total number of capillaries may predict overall disease outcome.

Some patients continue to have long-term problems including weakness (15%), rash (85%), and need for medications (35%). Luckily
most, if not all, appear to have good educational and vocational outcome with patients attending and finishing school and working.

Questions for Further Discussion
1. What medications are available for treatment of dermatomyositis?
2. What are the diagnostic criteria for dermatomyositis?
3. What consultants may be necessary for comprehensive care of a child with JDM?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MedlinePlus for these topics: Myositis and Muscle Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Pachman LM, Hayford JR, Chung A, et. al. Juvenile Dermatomyositis At Diagnosis Clinical Characeristics of 79 Children. J Rheumatology. 1998:25;1198-1204.

Huber AM, Lang B, LeBlanc CM, et. al. Medium- and Long-term Functional Outcomes in a Multicenter Cohort of Children with Juvenile Dermatomyositis. Arthritis Rheumatology 2000;43:541-49.

Ramanan AV, Feldman BM. Clinical Outcomes in Juvenile Dermatomyositis. Current Opinion in Rheumatology. 2002;14;658-662.

Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:854-856.

Lindsley CB. Juvenile Dermatomyositis Update. Current Rheumatology Reports. 2006:8;174-77.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.

    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement

    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    January 7, 2008