Dermatomyositis
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| Dermatomyositis Classification and external resources | |
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| X-Ray of the knee in a patient with dermatomyositis. | |
| ICD-10 | M33.0-M33.1 |
| ICD-9 | 710.3 |
| DiseasesDB | 10343 |
| MedlinePlus | 000839 |
| eMedicine | med/2608 derm/98 |
| MeSH | D003882 |
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US National Guidelines Clearinghouse on Dermatomyositis NICE Guidance on Dermatomyositis
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Overview
Dermatomyositis is connective-tissue disease that is characterized by inflammation of the muscles and the skin. Its cause is unknown, but it may result from either a viral infection or an autoimmune reaction. Up to 50% of the cases may be a paraneoplastic phenomenon, indicating the presence of cancer.
X-ray findings include dystrophic calcifications in the muscles.
There is a form of this disorder that strikes children, known as juvenile dermatomyositis.
"Gottron's papules", pink patches on the knuckles, and priapism, are associated with this disorder.
Pathology
The diagnosis of dermatomyositis can be confirmed by muscle biopsy. There are two classic microscopic findings of dermatomyositis. They are:
Microscopic findings
Cross sections of muscle reveal muscle fascicles with small, shrunken polygonal muscle fibers on the periphery of a fascicle surrounding central muscle fibers of normal, uniform size.
Aggregates of mature lymphocytes with small, dark nuclei and scant cytoplasm are seen surrounding vessels. Other inflammatory cells are distinctly uncommon. Immunohistochemistry can be used to demonstrate that both B- and T-cells are present in approximately equal numbers.[1] [1]
Mechanism
The mechanism is conjectured to be complement-mediated damage of microscopic vessels with muscle atrophy and lymphocytic inflammation secondary to tissue ischemia .
Diagnosis
History and Symptoms
Periorbital Heliotrope Rash (HELIOTROPE RASH: purple /erythematous rash affecting eyelids, malar, forehead and nasolabial folds), Gottron's papules at the knuckles (Purple /erythematous raised lesions over knuckles and extensor regions).
Physical Examination
Differential Diagnosis
Dermatomyositis must be differentiated from other common, lymphocyte predominant inflammatory myopathies. If present, the characteristic perifascicular atrophy makes this distinction trivial.
There is some overlap in the microscopic appearances of different inflammatory myopathies, but some helpful differences are often present. The rimmed vacuoles of inclusion body myositis (IBM) are absent in dermatomyositis. Polymyositis is characterised by diffuse or patchy inflammation of the muscle fascicles, a random pattern of muscle atrophy, and T-cell predominance with T-cells seen invading otherwise viable appearing muscle fibers.
Treatment
- 1. High Dose Prednisolone
- 2. Methotrexate (complication: may cause Interstitial lung disease)
- 3. IVIG
- 4. Azathioprine
- 5. Cyclophosphamide
References
External links
- The American College of Rheumatology's patient education page on myopathy
- Illustration of Gottron's papules
Muscular Dystrophy | |
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| The Nine Primary Muscular Dystrophies | Congenital • dystrophin (Becker's, Duchenne) • Distal • Emery-Dreifuss • Facioscapulohumeral • Limb-girdle muscular dystrophy • Myotonic • Oculopharyngeal |
| Related topics | |
| National/International Organizations | Muscular Dystrophy Association (USA) . Muscular Dystrophy Canada |
| US government Institutes and Legislation | NINDS • NIAMS • NICHD • MD CARE Act • Genetic Information Nondiscrimination Act • Americans with Disabilities Act of 1990 |
| National/International Events | Jerry Lewis MDA Telethon (USA) |
| Recent or Ongoing Clinical Trials | Stamulumab (MYO-029) |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
