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20-September-2008 10:21:22 - Dermatomyositis Dermatomyositis Classification and external resources 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 Dermatomyositis DM is a connective-tissue disease related to Polymyositis PM that is characterized by inflammation of the muscles and the skin. Contents 1 Causes 2 Prognosis 3 Presentation 4 Classification 5 Signs and symptoms 6 Pathology 7 Microscopic findings 8 Mechanism 9 Differential diagnosis 10 Treatment 11 Notable individuals with dermatomyositis 12 References 13 External links Causes The cause is unknown, but it may result from either a viral infection or an autoimmune reaction. Some cases of dermatomyositis actually overlap are combined with another autoimmune disease such as lupus, scleroderma, or vasculitis. Because of the link between DM and autoimmune disease, doctors and patients suspecting DM may find it helpful to run an ANA - antinuclear antibody - test, which in cases of a lupus-like nature may be positive usually from 1:160 to 1:640, with normal ranges at 1:40 and below. Some cases of DM are a paraneoplastic phenomenon, indicating the presence of cancer.1 In cases involving cancer, the cancer is usually pre-existent, with removal of the cancer resulting in remission of the DM. Prognosis Before the advent of modern treatments such as: prednisone, IVIG, plasmapheresis, chemotherapies, and other drugs, the mortality rate was very high with these conditions up to 75% of patients died in cases that involved the lungs. Now, in the 21st century, there are numerous treatments and immune-modulating drugs. Fortunately, over 90% of patients today will do well for many years, with remission being a possibility. However, it is still important that treatment begin as soon as possible. Presentation X-ray findings sometimes include dystrophic calcifications in the muscles, and patients may or may not notice small calcium deposits under the skin. Many do not have any calcium deposits of any kind. The rash also may come and go, and may not be dependent on the severity of the muscle involvement at the time. Gottron's papules, pink patches on the knuckles, and priapism, are associated with this disorder. Classification Dermatomyositis may be a type of autoimmune connective tissue disease.2 It is related to polymyositis and inclusion body myositis. There is a form of this disorder that strikes children, known as juvenile dermatomyositisJDM. For the most part Juvenile dermatomyositis is the same as the adult form, but the relationship with cancer is far lower, or non-existent. Signs and symptoms The main symptoms include skin rash and symmetric proximal muscle weakness which may be accompanied by pain. The pain may resemble the type experienced after strenuous exercise. Some DM patients have little pain, while in others esp. in JDM, the pain may be severe. It is important to remember that this condition varies from person to person in many ways. Skin findings occur in DM but not PM and are generally present at diagnosis. Gottron's sign is an erythematous, scaly eruption occurring in symmetric fashion over the MCP and interphalangeal joints can mimic psoriasis. Heliotrope rash is a violaceous eruption on the upper eyelids, often with swelling most specific, though uncommon. Shawl or V- sign is a diffuse, flat, erythematous lesion over the chest and shoulders or in a V over the anterior neck and chest, worsened with UV light. Erythroderma is a flat, erythematous lesion similar to the shawl sign but located in other areas, such as the malar region and the forehead. Periungual telangiectasias and erythema occur. Mechanic's hands also in PM refers to rough, cracked skin at the tips and lateral aspects of the fingers forming irregular dirty-appearing lines that resemble those seen in a laborer this is also associated with the anti-synthetase syndrome. Psoriaform changes in the scalp can occur. Centripetal flagellate erythema comprises linear, violaceous streaks on the trunk possibly caused by itching pruritic skin. Calcinosis cutis deposition of calcium in the skin is usually seen in juvenile DM, not adult DM. Dysphagia difficulty swallowing is another Pathology The diagnosis of dermatomyositis can be confirmed by muscle biopsy, EMG,and blood tests. On the muscle biopsy, there are two classic microscopic findings of dermatomyositis. They are: A mixed B- and T-cell perivascular inflammatory infiltrate Perifascicular muscle fiber atrophy Dermatomyositis is associated with autoantibodies, especially anti-Jo-1 antibody.3 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. Mechanism The mechanism is conjectured to be complement-mediated damage of microscopic vessels with muscle atrophy and lymphocytic inflammation secondary to tissue ischemia.4 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.5 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.1 Treatment Prednisolone Intravenous immunoglobulin Azathioprine Cyclophosphamide Rituximab6 Notable individuals with dermatomyositis English actor Sir Laurence Olivier 1907-89 contracted dermatomyositis in 1974 aged 67, and nearly died from the disease. Olivier suffered the effects for the rest of his life.citation needed Ricky Bell, the 1976 Heisman Trophy runner up and #1 pick in the 1977 NFL draft, died at age 29 from the disease.citation needed References ^ Scheinfeld NS 2006. Ulcerative paraneoplastic dermatomyositis secondary to metastatic breast cancer. Skinmed 5 2: 94-6. doi:10.1111/j.1540-9740.2006.03637.x. PMID 16603844. ^ Polymyositis and Dermatomyositis: Autoimmune Disorders of Connective Tissue: Merck Manual Home ion. ^ Ghirardello, A; Zampieri S, Tarricone E et al. May 2006. Clinical implications of autoantibody screening in patients with autoimmune myositis. Autoimmunity 39 3: 217-221. doi:10.1080/08916930600622645. PMID 16769655. ^ Benveniste, O; Squier W, Boyer O et al. Nov 2004. Pathogenesis of primary inflammatory myopathies. Presse Médicale 33 20: 1444-1450. doi:10.1016/S0755-49820498952-X. PMID 15611679. ^ Nirmalananthan, N; Holton JL, Hanna MG Nov 2004. Is it really myositis? A consideration of the differential diagnosis. Current Opinion in Rheumatology 16 6: 684-691. doi:10.1097/01.bor.0000143441.27065.bc. PMID 15577605. ^ Scheinfeld N 2006. A review of rituximab in cutaneous medicine. Dermatol. Online J. 12 1: 3. PMID 16638371. External links The Myositis Association 2 The American College of Rheumatology's patient education page on myopathy Illustration of Gottron's papules at University of Iowa v d e Diseases of the musculoskeletal system and connective tissue M, 710-739 Arthropathies Arthritis Septic arthritis - Reactive arthritis - Rheumatoid arthritis - Psoriatic arthritis - Felty syndrome - Juvenile idiopathic arthritis - Still's disease - crystal Gout, Chondrocalcinosis - Osteoarthritis Heberden's node, Bouchard's nodes - Monoarthritis/Polyarthritis Specific joints shoulder Winged scapula - elbow Cubitus valgus, Cubitus varus - hand Wrist drop, Boutonniere deformity, Swan neck deformity hip Protrusio acetabuli, Coxa valga, Coxa vara - leg Foot drop, Flat feet, Club foot, Unequal leg length - patella Luxating patella, Chondromalacia patellae - foot Bunion/hallux valgus, Hallux varus, Hallux rigidus, Hammer toe general terms Valgus deformity, Varus deformity Other Hemarthrosis - Arthralgia - Osteophyte - Hypermobility Systemic CT disorders vasculitis: Arteritis Polyarteritis nodosa, Takayasu's arteritis, Temporal arteritis - arterioles/capillaries Wegener's granulomatosis, Kawasaki disease, Churg-Strauss syndrome, Microscopic polyangiitis hypersensitivity/autoimmune: Hypersensitivity vasculitis - Goodpasture's syndrome - Systemic lupus erythematosus Drug-induced - Dermatomyositis Juvenile dermatomyositis - Polymyositis - Scleroderma - Sjögren's syndrome - Behçet's disease - Polymyalgia rheumatica - Eosinophilic fasciitis Dorsopathies spinal curvature Kyphosis, Lordosis, Scoliosis - Scheuermann's disease - Spondylolysis - Torticollis - Spondylolisthesis Spondylopathies Ankylosing spondylitis, Spondylosis, Spinal stenosis - Schmorl's nodes - Degenerative disc disease - Coccydynia - Back pain Radiculopathy, Neck pain, Sciatica, Low back pain Soft tissue disorders Muscle Myositis Pyomyositis - Myositis ossificans Fibrodysplasia ossificans progressiva Synovium and tendon Synovitis/Tenosynovitis Calcific tendinitis, Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome - Irritable hip - Ganglion cyst Bursa Bursitis Olecranon, Prepatellar, Trochanteric - Baker's cyst Fascia/fibroblastic Fasciitis Plantar fasciitis, Nodular fasciitis, Necrotizing fasciitis - Dupuytren's contracture - Fibromatosis Shoulder lesions Adhesive capsulitis - Rotator cuff tear - Subacromial bursitis Enthesis Enthesopathies Iliotibial band syndrome, Achilles tendinitis, Patellar tendinitis, Golfer's elbow, Tennis elbow, Metatarsalgia, Bone spur, Tendinitis Other, NEC Muscle weakness - Rheumatism - Myalgia - Neuralgia - Neuritis - Panniculitis - Fibromyalgia Osteopathies disorders of bone density and structure: Osteoporosis - Osteomalacia - continuity of bone Pseudarthrosis, Stress fracture - Monostotic fibrous dysplasia - Skeletal fluorosis - Aneurysmal bone cyst - Hyperostosis - Osteosclerosis Osteomyelitis - Avascular necrosis - Paget's disease of bone - Algoneurodystrophy - Osteolysis - Infantile cortical hyperostosis Chondropathies Juvenile osteochondrosis Legg-Calvé-Perthes syndrome, Osgood-Schlatter disease, Köhler disease, Sever's disease - Osteochondritis - Tietze's syndrome - Relapsing polychondritis See also congenital v d e Inflammation Acute Plasma derived mediators Bradykinin - complement C3, C5a, MAC - coagulation Factor XII, Plasmin, Thrombin Cell derived mediators preformed: Lysosome granules - vasoactive amines Histamine, Serotonin synthesized on demand: cytokines IFN-γ, IL-8, TNF-α, IL-1 - eicosanoids Leukotriene B4, Prostaglandins - Nitric oxide - Kinins Chronic Macrophage - Epithelioid cell - Giant cell - Granuloma Processes Traditional: Rubor - Calor - Tumor - Dolor - Functio laesa Modern: Acute-phase reaction - Chemotaxis - Fever - Pain - Vascular permeability - Vasodilation Specific types Cardiovascular Endocarditis - Myocarditis - Pericarditis - Vasculitis Arteritis, Phlebitis Digestive mouth Stomatitis, Gingivitis, Gingivostomatitis, Glossitis, Tonsillitis, Sialadenitis/Parotitis - tract Esophagitis, Gastritis, Gastroenteritis, Enteritis, Colitis, Enterocolitis, Duodenitis, Ileitis, Appendicitis, Proctitis - accessory Hepatitis, Cholangitis, Cholecystitis, Pancreatitis - Peritonitis Musc./int. Arthritis - Bursitis - Dermatitis Folliculitis - Dermatomyositis - Epicondylitis - Fasciitis - Fibrositis - Hidradenitis - Mastitis - Myositis - Osteitis - Synovitis - Tendinitis Nervous Encephalitis - Meningitis - Myelitis - Otitis - eye Blepharitis, Conjunctivitis, Dacryoadenitis, Iritis Respiratory upper Sinusitis, Rhinitis, Pharyngitis, Laryngitis - lower Tracheitis, Bronchitis, Bronchiolitis, Pneumonitis, Pleuritis Reproductive female: Cervicitis - Chorioamnionitis - Endometritis - Oophoritis - Salpingitis - Uveitis - Vaginitis - Vulvitis - Parametritis male: Epididymitis - Prostatitis - Orchitis newborn: Omphalitis Urinary Nephritis Glomerulonephritis, Pyelonephritis - Ureteritis - Cystitis - Urethritis v d e Paraneoplastic syndromes Mucocutaneous Dermatomyositis · Leser-Trélat sign · Acanthosis nigricans · Necrolytic migratory erythema · Sweet's syndrome · Pyoderma gangrenosum Neurological Paraneoplastic cerebellar degeneration · Encephalomyelitis · Limbic encephalitis · Opsoclonus · Polymyositis · Transverse myelitis · Lambert-Eaton myasthenic syndrome Hematological Granulocytosis Endocrine Hypercalcaemia · SIADH · Zollinger-Ellison syndrome Retrieved from http://en..org/wiki/Dermatomyositis Categories: Dermatology | Diseases involving the fasciae | Inflammations | Autoimmune diseasesHidden categories: All articles with statements | Articles with statements since April 2008 Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Italiano Nederlands 日本語 Polski This page was last modified on 12 August 2008, at 03:13
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