History

Fact Explanation
Introduction Dermatomyositis is an extremely rare connective tissue disorder and basically involves the muscles and skin. Rarely joints, oesophagus, lungs and heart also can be affected. The pathological process is mainly via idiopathic inflammation of the muscles with associated skin manifestations. Both children and adults can suffer from the illness. Patients with the illness usually presents with the skin manifestations and will have features of proximal muscle weakness with muscle involvement. Both muscle weakness and skin lesions could occur simultaneously or one may precede the other. [1][2]
Pathophysiology The entire pathophysiology of dermatomysitis is yet to be completed. Immune mediated inflammatory myopathy is the basic to the current knowledge. Inflammation is via activation of complement pathway and formation of membrane attack complex and other inflammatory mediators which causes inflammation and destruction of microvasculature of the muscles and the skin. This leads to microinfarction, perfascicular atrophy and necrosis of muscle tissue. Similar pathology is thought to be occurred in skin as well.[3][4][5]
Features of proximal muscle weakness This may precede, follow or occur concurrently with cutaneous manifestations. The mayopathy commonly affects proximal muscle groups and patient may complain difficulties in carrying out activities involving proximal muscle groups. They may complain the difficulty in standing from seated or squatting position, they might need support. They may feel difficulties when climbing stairs and mountains. Regarding the upper limb weakness they may feel the difficulty when reaching an object in a higher place, combing hair or taking of suits and similar activities. The muscles could be tender though it is not typical. In early child hood patient may develop tip toe gait due to contracture at ankle. [2][6][7]
Skin eruption mainly over light exposed areas Due to the above mentioned microinfarction and necrosis of the skin, the photo exposed area may get easy skin eruption. [8][9]
Skin eruption of other areas Eruption of the upper outer thigh, mid face, eye lid margins and dorsum of the hands would be typical areas. These lesions could be itchy and may be intense. [1][2][10]
Changes in fingers and nail folds Some times the patient may notice nail and finger changes which are typical for dermatomyositis which are described latter. [10[11]
Scaly scalp lesions and diffuse hair loss As in many connective tissue disorders this also could lead to hair loss. [10][12]
General symptoms such as low grade fever, loss of appetite, loss of weight, arthralgia and lethargy can occur Due to the general ill health and multi-systemic involvement generalized symptoms could occur. [1][2][13]
Difficulty in swallowing Due to the weak skeletal muscle of the oesophagus dysphagia can occur. [14][15]
Gastric reflux features Gastric and chest burn with regurgitation can occur due to sphincter malfunction due to weakness of the smooth muscles of the oesophagus. [14][16]
Features of heart failure Due to the involvement of cardiac muscles there may be patients with cardiomyopathy. Exertional dyspnoea, leg swelling and abdominal fullness may occur. [17][18]
Features of arrythmia Similarly due to the cardiac involvement varying types of arrythmia can occur causing palpitation, syncopal attacks. [19][20]
Features of respiratory difficulties Due to the involvement of respiratory muscles and interstitial lung involvement breathing difficulties could occur. [21]
Evidence of a malignancy Dermatomyositis could be a manifestation of an internal malignancy specially in adults. Therefore a thorough history should be taken in relation to evidence of concurrent or previous malignant diseases. [22][23][24]
References
  1. SHRESTHA S, WERSHIL B, SARWARK JF, NIEWOLD TB, PHILIPP T, PACHMAN LM. Lesional and Nonlesional Skin From Patients With Untreated Juvenile Dermatomyositis Displays Increased Numbers of Mast Cells and Mature Plasmacytoid Dendritic Cells Arthritis Rheum [online] 2010 Sep, 62(9):2813-2822 [viewed 09 December 2014] Available from: doi:10.1002/art.27529
  2. MARVI U, CHUNG L, FIORENTINO DF. Clinical Presentation and Evaluation of Dermatomyositis Indian J Dermatol [online] 2012, 57(5):375-381 [viewed 09 December 2014] Available from: doi:10.4103/0019-5154.100486
  3. GREENBERG SA. Dermatomyositis and Type 1 Interferons Curr Rheumatol Rep [online] 2010 Jun, 12(3):198-203 [viewed 09 December 2014] Available from: doi:10.1007/s11926-010-0101-6
  4. SALAJEGHEH M, KONG SW, PINKUS JL, WALSH RJ, LIAO A, NAZARENO R, AMATO AA, KRASTINS B, MOREHOUSE C, HIGGS BW, JALLAL B, YAO Y, SARRACINO DA, PARKER K, GREENBERG SA. Interferon-Stimulated Gene 15 (ISG15) Conjugates Proteins in Dermatomyositis Muscle with Perifascicular Atrophy Ann Neurol [online] 2010 Jan, 67(1):53-63 [viewed 09 December 2014] Available from: doi:10.1002/ana.21805
  5. BAECHLER EC, BILGIC H, REED AM. Type I interferon pathway in adult and juvenile dermatomyositis Arthritis Res Ther [online] 2011, 13(6):249 [viewed 09 December 2014] Available from: doi:10.1186/ar3531
  6. CASTRO C, GOURLEY M. Diagnosis and treatment of inflammatory myopathy: issues and management Ther Adv Musculoskelet Dis [online] 2012 Apr, 4(2):111-120 [viewed 09 December 2014] Available from: doi:10.1177/1759720X11425092
  7. CHAWLA J. Stepwise Approach to Myopathy in Systemic Disease Front Neurol [online] :49 [viewed 09 December 2014] Available from: doi:10.3389/fneur.2011.00049
  8. CRUELLAS MG, DOS SANTOS TRINDADE VIANA V, LEVY-NETO M, DE SOUZA FH, SHINJO SK. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis Clinics (Sao Paulo) [online] 2013 Jul, 68(7):909-914 [viewed 09 December 2014] Available from: doi:10.6061/clinics/2013(07)04
  9. KIM JE, JEONG MG, LEE HE, KO JY, RO YS. Successful Treatment of Cutaneous Lesions of Dermatomyositis with Topical Pimecrolimus Ann Dermatol [online] 2011 Aug, 23(3):348-351 [viewed 09 December 2014] Available from: doi:10.5021/ad.2011.23.3.348
  10. MADKE B, NAYAK C. Eponymous signs in dermatology Indian Dermatol Online J [online] 2012, 3(3):159-165 [viewed 10 December 2014] Available from: doi:10.4103/2229-5178.101810
  11. ELMANSOUR I, CHIHEB S, BENCHIKHI H. Nail changes in connective tissue diseases: a study of 39 cases Pan Afr Med J [online] :150 [viewed 10 December 2014] Available from: doi:10.11604/pamj.2014.18.150.4637
  12. LEVY LL, EMER JJ. Female pattern alopecia: current perspectives Int J Womens Health [online] :541-556 [viewed 10 December 2014] Available from: doi:10.2147/IJWH.S49337
  13. RAFAILIDIS PI, KAPASKELIS A, FALAGAS ME. Periorbital and facial swelling due to dermatomyositis CMAJ [online] 2007 May 22, 176(11):1580-1581 [viewed 10 December 2014] Available from: doi:10.1503/cmaj.061205
  14. JOSHI D, MAHMOOD R, WILLIAMS P, KITCHEN P. Dysphagia secondary to dermatomyositis treated successfully with intravenous immunoglobulin: a case report Int Arch Med [online] :12 [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-12
  15. KUSANO J, TAKAHASHI Y, MISAKI Y, MURAI N. Adult Dermatomyositis with Bleeding Ulcer in the Pharynx Case Rep Otolaryngol [online] 2014:854841 [viewed 10 December 2014] Available from: doi:10.1155/2014/854841
  16. COJOCARU M, COJOCARU IM, SILOSI I, VRABIE CD. Gastrointestinal Manifestations in Systemic Autoimmune Diseases Maedica (Buchar) [online] 2011 Jan, 6(1):45-51 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3150032
  17. AFZAL A, HIGGINS R, PHILBIN E. Heart transplant for dilated cardiomyopathy associated with polymyositis Heart [online] 1999 Oct, 82(4):e4 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1760297
  18. LINOS E, FIORENTINO D, LINGALA B, KRISHNAN E, CHUNG L. Atherosclerotic cardiovascular disease and dermatomyositis: an analysis of the Nationwide Inpatient Sample survey Arthritis Res Ther [online] 2013, 15(1):R7 [viewed 10 December 2014] Available from: doi:10.1186/ar4135
  19. DHOBLE A, PUTTARAJAPPA C, NEIBERG A. Dermatomyositis and supraventricular tachycardia Int Arch Med [online] :25 [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-25
  20. SéNéCHAL M, CRêTE M, COUTURE C, POIRIER P. Myocardial dysfunction in polymyositis Can J Cardiol [online] 2006 Aug, 22(10):869-871 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2569021
  21. PLOWMAN PN, STABLEFORTH DE. Dermatomyositis with fibrosing alveolitis: response to treatment with cyclophosphamide. Proc R Soc Med [online] 1977 Oct, 70(10):738-740 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1543423
  22. STOCKTON D, DOHERTY VR, BREWSTER DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study Br J Cancer [online] 2001 Jul, 85(1):41-45 [viewed 10 December 2014] Available from: doi:10.1054/bjoc.2001.1699
  23. TAMBE SA, JERAJANI HR. Dermatomyositis associated with malignancy: A report of 3 cases Indian Dermatol Online J [online] 2013, 4(4):326-332 [viewed 10 December 2014] Available from: doi:10.4103/2229-5178.120664
  24. PELOSOF LC, GERBER DE. Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment Mayo Clin Proc [online] 2010 Sep, 85(9):838-854 [viewed 10 December 2014] Available from: doi:10.4065/mcp.2010.0099

Examination

Fact Explanation
Dermatological manifestation: Heliotrope Rash This is one of the pathognomonic cutaneous lesion of dermatomyositis. It involves the periorbital skin simultaneously. It is typically a violaceous to erythematous rash which could be usually associated with itching and swelling. [1][2][3][4]
Dermatological manifestation: Gottron papules Gottron papules are also another pathognomonic lesion in dermatomyositis involving the bony prominences specially those of hands (metacarpophalengeal and interphalengeal joints). It is a flat-topped, erythematous to violaceous papules which may resemble psoriasis or lesions in lupus erythromatosus. [1][2][3][4]
Dermatological manifestation: Photo sensitive rashes (poikiloderma) in photo exposed areas Poikiloderma has been labeled for associated changes in pigmentation, telangiactesis and erythema of the skin which can be more obvious over photo exposed areas. According to the different areas of the lesion different names have been given as physical signs. The upper back (shawl sign), lateral thighs (holster sign) and upper chest and arm in 'V' configuration. Photoexposed areas could be eruptive and itchy even. [1][5][6]
Dermatological manifestation: Mechanic's hands This consists of rough, cracked skin at the tips and lateral aspects of the fingers creating irregular dirty-appearing markings that mimic those in a mechanic. This sign is also seen in polymyositis. Presence of mechanic's hand as an association with high prevalence of lung diseases as well. [7][8]
Dermatological manifestation: Calcinosis Calcinosis of soft tissues could occur in paediatric patients with the disease which could be obvious at finger tips as well as other areas of the skin.[9][10]
Dermatological manifestation: Raynods phenomenon Bluish discoloration of the extremities when exposed cold is characterized as Raynods phenomenon and also can be seen in connective tissue disorders including dermatomyositis. [4][11]
Manifestation of skeletal muscle weakness Mainly the proximal muscles are affected with preserved distal muscle strength. Extensors of the upper limb are commonly affected. Proximal muscle power will be reduced and there may be difficulties in standing from seated or squatting position, combing and similar activities. There may be weak trunk muscles as well. Sensation is preserved and tendon reflexes may be normal unless severely affected. During early phase of the illness muscles would be tender. [12][13][14]
Cardiac involvement with features of heart failure Due to the cardiac muscle involvement and cardiomayopathy cardiac failure may follow. Ankle edema, ascites, increased Jugular Venous Pressure, pulmonary edema and 3rd heart soud may be obvious. If arrythmias exist pulse may be irregular. [15][16]
Joint involvement Poly arthritis may occur with evidence of inflammation mainly involving the small joints of the hand. [17]
Evidence of malignancy Thorough systemic examination should carried out to detect hidden malignancies and evidence of secondaries and lymph nodes as dermatomyositis could be a manifestation of a malignancy. [18][19]
References
  1. KIM JE, JEONG MG, LEE HE, KO JY, RO YS. Successful Treatment of Cutaneous Lesions of Dermatomyositis with Topical Pimecrolimus Ann Dermatol [online] 2011 Aug, 23(3):348-351 [viewed 10 December 2014] Available from: doi:10.5021/ad.2011.23.3.348
  2. KLEIN R, ROSENBACH M, KIM E, KIM B, WERTH V, DUNHAM J. Tumor necrosis factor-inhibitor associated dermatomyositis Arch Dermatol [online] 2010 Jul, 146(7):780-784 [viewed 10 December 2014] Available from: doi:10.1001/archdermatol.2010.142
  3. KHAMBATTA S, WITTICH CM. Amyopathic Dermatomyositis Mayo Clin Proc [online] 2010 Nov, 85(11):e82 [viewed 10 December 2014] Available from: doi:10.4065/mcp.2010.0514
  4. MARVI U, CHUNG L, FIORENTINO DF. Clinical Presentation and Evaluation of Dermatomyositis Indian J Dermatol [online] 2012, 57(5):375-381 [viewed 10 December 2014] Available from: doi:10.4103/0019-5154.100486
  5. SAGI L, AMICHAI B, BARZILAI A, SHPIRO D, BAUM S, NEIMUSHIN A, LIDAR M, TRAU H. Dermatomyositis and small cell carcinoma of the bladder Can Fam Physician [online] 2009 Oct, 55(10):997-999 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2762298
  6. LOVE LA, WEINBERG CR, MCCONNAUGHEY DR, ODDIS CV, MEDSGER TA JR, REVEILLE JD, ARNETT FC, TARGOFF IN, MILLER FW. Ultraviolet Radiation Intensity Predicts the Relative Distribution of Dermatomyositis and Anti-Mi-2 Autoantibodies in Women Arthritis Rheum [online] 2009 Aug, 60(8):2499-2504 [viewed 10 December 2014] Available from: doi:10.1002/art.24702
  7. SOHARA E, SARAYA T, SATO S, TSUJIMOTO N, WATANABE T, TAKATA S, TANAKA Y, ISHII H, TAKIZAWA H, GOTO H. Mechanic’s hands revisited: is this sign still useful for diagnosis in patients with lung involvement of collagen vascular diseases? BMC Res Notes [online] :303 [viewed 10 December 2014] Available from: doi:10.1186/1756-0500-7-303
  8. TAGGART AJ, FINCH MB, COURTNEY PA, GORMLEY GJ. Anti Jo-1 myositis. 'Mechanic's hands' and interstitial lung disease. Ulster Med J [online] 2002 May, 71(1):68-71 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2475351
  9. JAYANTHI MR, BASHER RK, BHADADA SK, BHATTACHARYA A, MITTAL BR. A case of adult dermatomyositis with calcinosis universalis Indian J Nucl Med [online] 2014, 29(4):278-279 [viewed 10 December 2014] Available from: doi:10.4103/0972-3919.142649
  10. LEE GL, ANETAKIS POULOS G, J. ZIRWAS M. Dermatomyositis and Metastatic Lung Adenocarcinoma First Presenting as Calcinosis Cutis J Clin Aesthet Dermatol [online] 2012 Jan, 5(1):47-48 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3277092
  11. FIORENTINO D, CASCIOLA-ROSEN L. TIF1 autoantibodies in dermatomyositis shed insight into the cancer-myositis connection Arthritis Rheum [online] 2012 Feb, 64(2):346-349 [viewed 10 December 2014] Available from: doi:10.1002/art.33402
  12. ZHAO Y, FEDCZYNA TO, MCVICKER V, CALIENDO J, LI H, PACHMAN LM. Apoptosis in the Skeletal Muscle of Untreated Children with Juvenile Dermatomyositis: Impact of Duration of Untreated Disease Clin Immunol [online] 2007 Nov, 125(2):165-172 [viewed 10 December 2014] Available from: doi:10.1016/j.clim.2007.06.011
  13. COLEY W, RAYAVARAPU S, PANDEY GS, SABINA RL, VANDER MEULEN JH, AMPONG B, WORTMANN RL, RAWAT R, NAGARAJU K. The molecular basis of skeletal muscle weakness in a mouse model of inflammatory myopathy Arthritis Rheum [online] 2012 Nov, 64(11):3750-3759 [viewed 10 December 2014] Available from: doi:10.1002/art.34625
  14. ENGLUND P, LINDROOS E, NENNESMO I, KLARESKOG L, LUNDBERG IE. Skeletal Muscle Fibers Express Major Histocompatibility Complex Class II Antigens Independently of Inflammatory Infiltrates in Inflammatory Myopathies Am J Pathol [online] 2001 Oct, 159(4):1263-1273 [viewed 10 December 2014] Available from: doi:10.1016/S0002-9440(10)62513-8
  15. AFZAL A, HIGGINS R, PHILBIN E. Heart transplant for dilated cardiomyopathy associated with polymyositis Heart [online] 1999 Oct, 82(4):e4 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1760297
  16. LINOS E, FIORENTINO D, LINGALA B, KRISHNAN E, CHUNG L. Atherosclerotic cardiovascular disease and dermatomyositis: an analysis of the Nationwide Inpatient Sample survey Arthritis Res Ther [online] 2013, 15(1):R7 [viewed 10 December 2014] Available from: doi:10.1186/ar4135
  17. CRUELLAS MG, DOS SANTOS TRINDADE VIANA V, LEVY-NETO M, DE SOUZA FH, SHINJO SK. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis Clinics (Sao Paulo) [online] 2013 Jul, 68(7):909-914 [viewed 10 December 2014] Available from: doi:10.6061/clinics/2013(07)04
  18. ANTIOCHOS BB, BROWN LA, LI Z, TOSTESON TD, WORTMANN RL, RIGBY WF. Malignancy Is Associated with Dermatomyositis But Not Polymyositis in Northern New England, USA J Rheumatol [online] 2009 Dec, 36(12):10.3899/jrheum.090549 [viewed 10 December 2014] Available from: doi:10.3899/jrheum.090549
  19. TAMBE SA, JERAJANI HR. Dermatomyositis associated with malignancy: A report of 3 cases Indian Dermatol Online J [online] 2013, 4(4):326-332 [viewed 10 December 2014] Available from: doi:10.4103/2229-5178.120664

Differential Diagnoses

Fact Explanation
Polymyositis Almost similar pathophysiology leads two similar disorders called polymyositis and dermatomyositis. In dermato myositis the muscle involvement is prominent with less dermatological manifestations. Pathognomonic signs such as Heliotrope rash and Gottron rash won't be present in polymyositis. [1][2]
Inflammatory myopathies, motor neuron disease, myasthenia gravis, muscular dystrophies, inherited, metabolic, drug–induced, endocrine, and infectious myopathies. Other myopathies as mentioned should be distinguished from dermatomyositis. Non of the pathognomonic skin lesions such as Heliotrpe rash or Gottron rash is present in above myopathies. Other than that the severity of the muscle weakness is mild and less significant in dermatomyositis compared to the others. [3][4][5]
Inclusion body myositis Typical skin lesions are absent. Other than that the muscle weakness is mostly distal and more insidious than in dermatomyositis. MRI supports the diagnosis and histology shows typical inclusion bodies. [6]
Drug induced myopathy Careful history should be elicited regarding exposure to causative medications such as corticosteroids, statins, antivirals, antiretrovirals, alcohol, colchicine and cocaine. If doubtful electromyography and muscle biopsy can be performed. [5][7]
Hypothyroidism induced myopathy Hypothyroidism could cause similar proximal mayopathy. In the absence of other features of dermatomyositis and with the presence of other features of hypothyroidism including delayed relaxation phase of the deep tendon reflexes the hypothyroidism can be suspected. Thyroid function tests would be suggestive and biopsy would not show inflammation of the muscle. [5][7]
Muscle dystrophy Muscle dystrophies are inherited muscle disorders due to defects of genes needed for normal muscular function. In controversies biopsy will distinguish two types of pathologies. [8][9]
Systemic Lupus Erethromatosus (SLE) Some eruptive and photosensitive skin reactions of dermatomyositis may resemble that of SLE. But typical signs such as Heliotrpe and Gottron rash may be absent. Other than that the midfacial lesion does not spare the nasolabial folds in dermatomyositis. Can be differentiated by assessing the severity of muscle involvement, organ involvement and serology (ANA, Ds-DNA). [10][11]
Psoriasis Unlike in dermatomyositis there would be thicker and silvery without any evidence of pathognomonic lesions of dermatomyositis. Photosensitive poikiloderma is absent with out involvement of musculature. [12][13]
Seborrheic dermatitis Greasy yellow scalp scales are present without other skin or systemic involvement. [12][13]
References
  1. KIKUCHI Y, KOARADA S, TADA Y, USHIYAMA O, MORITO F, SUZUKI N, OHTA A, HORIUCHI T, MIYAKE K, NAGASAWA K. Difference in B cell activation between dermatomyositis and polymyositis: analysis of the expression of RP105 on peripheral blood B cells Ann Rheum Dis [online] 2001 Dec, 60(12):1137-1140 [viewed 10 December 2014] Available from: doi:10.1136/ard.60.12.113
  2. RAYCHAUDHURI SP, MITRA A. Polymyositis and dermatomyositis: Disease spectrum and classification Indian J Dermatol [online] 2012, 57(5):366-370 [viewed 10 December 2014] Available from: doi:10.4103/0019-5154.100477
  3. SERRATRICE G. Clinical semiology of neuromuscular diseases (5) Acta Myol [online] 2008 Oct, 27(2):45-48 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2858937
  4. KRAKER J, ŽIVKOVIć SA. Autoimmune Neuromuscular Disorders Curr Neuropharmacol [online] 2011 Sep, 9(3):400-408 [viewed 10 December 2014] Available from: doi:10.2174/157015911796558000
  5. VALIYIL R, CHRISTOPHER-STINE L. Drug-related Myopathies of Which the Clinician Should Be Aware Curr Rheumatol Rep [online] 2010 Jun, 12(3):213-220 [viewed 10 December 2014] Available from: doi:10.1007/s11926-010-0104-3
  6. IKEZOE K, OHSHIMA S, OSOEGAWA M, TANAKA M, OGAWA K, NAGATA K, KIRA J. Expression of granulysin in polymyositis and inclusion‐body myositis J Neurol Neurosurg Psychiatry [online] 2006 Oct, 77(10):1187-1190 [viewed 10 December 2014] Available from: doi:10.1136/jnnp.2005.081810
  7. CHAWLA J. Stepwise Approach to Myopathy in Systemic Disease Front Neurol [online] :49 [viewed 10 December 2014] Available from: doi:10.3389/fneur.2011.00049
  8. DE PASQUALE L, D'AMICO A, VERARDO M, PETRINI S, BERTINI E, DE BENEDETTI F. Increased muscle expression of interleukin-17 in Duchenne muscular dystrophy Neurology [online] 2012 Apr 24, 78(17):1309-1314 [viewed 10 December 2014] Available from: doi:10.1212/WNL.0b013e3182518302
  9. SHINJO SK, SALLUM AM, SILVA CA, MARIE SK. Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis Clinics (Sao Paulo) [online] 2012 Aug, 67(8):885-890 [viewed 10 December 2014] Available from: doi:10.6061/clinics/2012(08)05
  10. KAMENARSKA Z, DZHEBIR G, HRISTOVA M, SAVOV A, VINKOV A, KANEVA R, MITEV V, DOURMISHEV L. IL-1RN VNTR Polymorphism in Adult Dermatomyositis and Systemic Lupus Erythematosus Dermatol Res Pract [online] 2014:953597 [viewed 10 December 2014] Available from: doi:10.1155/2014/953597
  11. MCDONALD CJ, TURNER RW. Lupus Erythematosus, Systemic Scleroderma and Dermatomyositis J Natl Med Assoc [online] 1965 Jan, 57(1):26-31 [viewed 10 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2610692
  12. MADKE B, NAYAK C. Eponymous signs in dermatology Indian Dermatol Online J [online] 2012, 3(3):159-165 [viewed 10 December 2014] Available from: doi:10.4103/2229-5178.101810
  13. ALSAAD KO, GHAZARIAN D. My approach to superficial inflammatory dermatoses J Clin Pathol [online] 2005 Dec, 58(12):1233-1241 [viewed 10 December 2014] Available from: doi:10.1136/jcp.2005.027151

Investigations - for Diagnosis

Fact Explanation
Basic blood investigations and ECG Full blood count might show mild anaemia which is usually due to anaemia of chronic disease. Other inflammatory markers such as ESR and CRP will be elevated. ECG will show the associated arrhythmia and features of ventricular dysfunction. [1][2]
Electromyography Electromyography will show characteristic findings for inflammatory myopathy tho it is non specific and not diagnostic. [3][4]
Muscle enzyme levels Most of the time muscle enzyme levels would be elevated. Main muscle enzyme which is widely available is creatine kinase level which is more sensitive and more specific. Other enzyme levels usually altered are aldolase, aspartate aminotransferase and lactate dehydrogenase. [5][6][7][8]
Serological investigations Though the serology is not routinely used for diagnosis those can be performed for classification to subtypes and for prognostification. These antibodies are generally called as myositis specific antibodies (MSA). Anti nuclear antibody (ANA) will be positive in most patients though it is not essential for the diagnosis. Other documented MSA auto anti bodies are Anti-Mi-02 and Anti-Jo-01. [9][10][11][12]
Imaging Imaging would start from simple chest x-ray joint x ray. Chest xray may show associated interstitial lung diseases, cardiomegaly with cardiac involvement, features of heart failure, features of a malignant association such as pleural effusion, malignant pulmonary shadows and lymphadenopathy. Join xray may show Ultra sound scan of the muscle can be done to evaluate the status of muscle. Ultrasound of the neck, chest and abdomen can be performed to see undetected malignancies and lymph nodes which may be associated with the disease. Trans vaginal scan would be helpful in detecting gynaecological malignancies and ultrasound breast and mammography has to be performed if a breast cancer is suspected. CT scan of the neck , thorax, abdomen and pelvis has to be carried out if a malignancy is highly suspected. MRI scan of the affected muscles would be beneficial in evaluating the muscle inflammation and for differential diagnosis. [8][13][14][15]
Skin and Muscle biopsy for histological evaluation Biopsy can be performed to aid the diagnosis, for differential diagnosis and for detecting steroid induced myopathy. Skin biopsy would show interface dermatitis which is similar to lupus erythematosus. Muscle biopsy can be performed by a needle or as an open biopsy. Muscle biopsy is diagnostic and will show perivascular and interfascicular inflammatory infiltrates with associated degeneration of muscle fibers. [16][17][18]
References
  1. LAURET EUGENIA, RODRIGO LUIS. Celiac Disease and Autoimmune-Associated Conditions. BioMed Research International [online] 2013 December, 2013:1-17 [viewed 10 December 2014] Available from: doi:10.1155/2013/127589
  2. DHOBLE ABHIJEET, PUTTARAJAPPA CHETHAN, NEIBERG ALAN. Dermatomyositis and supraventricular tachycardia. Array [online] 2008 December [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-25
  3. PRESLEY BRADLEY, BUSH JEFFREY, WATSON SIMON. Dermatomyositis with Extensive Calcification in an Adult. WestJEM [online] 2012 February, 13(1):136-138 [viewed 10 December 2014] Available from: doi:10.5811/westjem.2011.8.6823
  4. ORTIGOSA LUCIENA CEGATTO MARTINS, REIS VITOR MANUEL SILVA DOS. Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil. An. bras. dermatol. [online] 2014 September, 89(5):719-727 [viewed 10 December 2014] Available from: doi:10.1590/abd1806-4841.20143422
  5. VOLOCHAYEV RITA. Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups. TORJ [online] 2012 June, 6(1):54-63 [viewed 10 December 2014] Available from: doi:10.2174/1874312901206010054
  6. VALIYIL RITU, CHRISTOPHER-STINE LISA. Drug-related Myopathies of Which the Clinician Should Be Aware. Curr Rheumatol Rep [online] December, 12(3):213-220 [viewed 10 December 2014] Available from: doi:10.1007/s11926-010-0104-3
  7. CHAWLA JASVINDER. Stepwise Approach to Myopathy in Systemic Disease. Front. Neur. [online] 2011 December [viewed 10 December 2014] Available from: doi:10.3389/fneur.2011.00049
  8. KLEIN RACHEL, ROSENBACH MISHA, KIM ELLEN J., KIM BRIAN, WERTH VICTORIA P., DUNHAM JONATHAN. Tumor Necrosis Factor Inhibitor–Associated Dermatomyositis. Arch Dermatol [online] 2010 July [viewed 10 December 2014] Available from: doi:10.1001/archdermatol.2010.142
  9. CRUELLAS MG, VIANA VS, LEVY-NETO M, SOUZA FH, SHINJO SK. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clinics [online] 2013 July, 68(7):909-914 [viewed 10 December 2014] Available from: doi:10.6061/clinics/2013(07)04
  10. WEDDERBURN LUCY R., RIDER LISA G.. Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatment. Best Practice & Research Clinical Rheumatology [online] 2009 October, 23(5):665-678 [viewed 10 December 2014] Available from: doi:10.1016/j.berh.2009.07.007
  11. ORTIGOSA LUCIENA CEGATTO MARTINS, REIS VITOR MANUEL SILVA DOS. Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil. An. bras. dermatol. [online] 2014 September, 89(5):719-727 [viewed 10 December 2014] Available from: doi:10.1590/abd1806-4841.20143422
  12. KRAKER JESSICA, A. ZIVKOVIC SASA. Array. CN [online] 2011 September, 9(3):400-408 [viewed 10 December 2014] Available from: doi:10.2174/157015911796558000
  13. FATHI M. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Annals of the Rheumatic Diseases [online] 2004 March, 63(3):297-301 [viewed 10 December 2014] Available from: doi:10.1136/ard.2003.006122
  14. PELOSOF LORRAINE C., GERBER DAVID E.. Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment. Mayo Clinic Proceedings [online] 2010 September, 85(9):838-854 [viewed 10 December 2014] Available from: doi:10.4065/mcp.2010.0099
  15. MCCREA NADINE, AL-SABBAGH AFRAA, AHMED SOLIMAN, WALKER DAVID, RANGARAJ SATYAPAL. Onset of juvenile dermatomyositis following varicella infection in a 12-month-old child: a case report. Array [online] 2014 December [viewed 10 December 2014] Available from: doi:10.1186/1752-1947-8-54
  16. SHRESTHA SHEELA, WERSHIL BARRY, SARWARK JOHN F., NIEWOLD TIMOTHY B., PHILIPP TERESA, PACHMAN LAUREN M.. Lesional and nonlesional skin from patients with untreated juvenile dermatomyositis displays increased numbers of mast cells and mature plasmacytoid dendritic cells. Arthritis & Rheumatism [online] December, 62(9):2813-2822 [viewed 10 December 2014] Available from: doi:10.1002/art.27529
  17. GREENBERG STEVEN A.. Dermatomyositis and Type 1 Interferons. Curr Rheumatol Rep [online] December, 12(3):198-203 [viewed 10 December 2014] Available from: doi:10.1007/s11926-010-0101-6
  18. FIORENTINO DAVIDF, MARVI UMAIMA, CHUNG LORINDA. Clinical presentation and evaluation of dermatomyositis. Indian J Dermatol [online] 2012 December [viewed 10 December 2014] Available from: doi:10.4103/0019-5154.100486

Investigations - for Management

Fact Explanation
Full blood count During the management FBC will show the associated anaemia which is usually due to anaemia of chronic disease and iron deficiency anaemia. This has to corrected promptly as it can aggravate the existing cardiac failure. Increased white cell count will be elevated in case of infection which has to be treated accordingly because the managing immunosuppressants could cause infection. [1][2]
Inflammatory markers (ESR/CRP) These would be elevated in disease flair up and with ongoing infection. So the detection of them can be made. [3][4]
ECG ECG should be monitored during the course of treatment because it shows the status of associated arrhythmias. [2]
2D Echo cardiogram As cardiomayopathy can be associated with the illness follow up 2D Echo should be performed to see the cardiac status. [5]
Muscle enzymes During flair up of the disease the muscle enzymes specially creatine kinase level will be elevated.[6][7]
Imaging and malignant markers for detecting associated malignancy Imaging and malignant markers such as CA-125, CEA, CA-19.9 should be carried out annually for 3 years in adult patients. [8][9]
Liver function tests,renal function tests and urine full report Serum creatine, AST, ALT and UFR has to be performed to see occurrence of renal or liver impairment which could be due to the concurrent other auto immune pathologies or due to the treatment.[10]
References
  1. LAURET EUGENIA, RODRIGO LUIS. Celiac Disease and Autoimmune-Associated Conditions. BioMed Research International [online] 2013 December, 2013:1-17 [viewed 10 December 2014] Available from: doi:10.1155/2013/127589
  2. DHOBLE ABHIJEET, PUTTARAJAPPA CHETHAN, NEIBERG ALAN. Dermatomyositis and supraventricular tachycardia. Array [online] 2008 December [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-25
  3. PELOSOF LORRAINE C., GERBER DAVID E.. Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment. Mayo Clinic Proceedings [online] 2010 September, 85(9):838-854 [viewed 10 December 2014] Available from: doi:10.4065/mcp.2010.0099
  4. CRUELLAS MG, VIANA VS, LEVY-NETO M, SOUZA FH, SHINJO SK. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clinics [online] 2013 July, 68(7):909-914 [viewed 10 December 2014] Available from: doi:10.6061/clinics/2013(07)04
  5. LINOS ELENI, FIORENTINO DAVID, LINGALA BHARATHI, KRISHNAN ESWAR, CHUNG LORINDA. Atherosclerotic cardiovascular disease and dermatomyositis: an analysis of the Nationwide Inpatient Sample survey. Array [online] 2013 December [viewed 10 December 2014] Available from: doi:10.1186/ar4135
  6. VOLOCHAYEV RITA. Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups. TORJ [online] 2012 June, 6(1):54-63 [viewed 10 December 2014] Available from: doi:10.2174/1874312901206010054
  7. VALIYIL RITU, CHRISTOPHER-STINE LISA. Drug-related Myopathies of Which the Clinician Should Be Aware. Curr Rheumatol Rep [online] December, 12(3):213-220 [viewed 10 December 2014] Available from: doi:10.1007/s11926-010-0104-3
  8. KLEIN RACHEL, ROSENBACH MISHA, KIM ELLEN J., KIM BRIAN, WERTH VICTORIA P., DUNHAM JONATHAN. Tumor Necrosis Factor Inhibitor–Associated Dermatomyositis. Arch Dermatol [online] 2010 July [viewed 10 December 2014] Available from: doi:10.1001/archdermatol.2010.142
  9. PELOSOF LORRAINE C., GERBER DAVID E.. Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment. Mayo Clinic Proceedings [online] 2010 September, 85(9):838-854 [viewed 10 December 2014] Available from: doi:10.4065/mcp.2010.0099
  10. VOLOCHAYEV RITA. Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups. TORJ [online] 2012 June, 6(1):54-63 [viewed 10 December 2014] Available from: doi:10.2174/1874312901206010054

Management - General Measures

Fact Explanation
Health education The patient should be aware about the disease including the rarity of it. Patient should know the prognosis, disease progress, avoidance factors, treatment modalities and side effects of long term treatment. Not only the patient all the family members with the care givers should participate in the discussion as their support is more important to the patient. [1]
Avoidance of exposure to sunlight Photo sensitivity rashes may be distressing to the patient. So advice should be given regarding the avoidance of sunlight and applying sunscreens.Photo protective clothing is useful.[2][3]
Adequate rest When the muscles are severely inflamed and when the cardiac failure with cardiomyopathy is associated adequate rest should be there.[4]
Physiotherapy and rehabilitation Physiotherapy with regular muscle exercise would help in preventing contracture formation and maintaining the muscle strength as much as possible according to the severity. Rehabilitation would help in carrying out day to day activities and mobilizing in disabled patients. Regular physiotherapy would also help in maintaining bone integrity as these patients are highly vulnerable for osteoporosis due to long term steroids. Any how the vigorous training should be better avoided. [1][5][6]
Measures to prevent Gastro Oesophageal Reflux Disease The patient should take light meals and the dinner should be adequately apart from the sleeping time. The head end should be little propped up. Proton pump inhibitors and anti emetics can be used if persistently symptomatic.[7]
Dietary modification The patients should take a balanced diet and a high protein diet should be recommended if there is a significant loss.[8]
References
  1. MARTIN N., LI C. K., WEDDERBURN L. R.. Juvenile dermatomyositis: new insights and new treatment strategies. Therapeutic Advances in Musculoskeletal Disease [online] December, 4(1):41-50 [viewed 10 December 2014] Available from: doi:10.1177/1759720X11424460
  2. NISTALA KIRAN, WEDDERBURN LUCY R.. Update in juvenile myositis. Current Opinion in Rheumatology [online] 2013 November, 25(6):742-746 [viewed 10 December 2014] Available from: doi:10.1097/01.bor.0000434674.62644.02
  3. GRöNHAGEN CARINAM, NYBERG FILIPPA. Cutaneous lupus erythematosus: An update. Indian Dermatol Online J [online] 2014 December [viewed 10 December 2014] Available from: doi:10.4103/2229-5178.126020
  4. ALEMO MUNTERS LI, DASTMALCHI MARYAM, KATZ ABRAM, ESBJöRNSSON MONA, LOELL INGELA, HANNA BALSAM, LIDéN MARIA, WESTERBLAD HåKAN, LUNDBERG INGRID E, ALEXANDERSON HELENE. Improved exercise performance and increased aerobic capacity after endurance training of patients with stable polymyositis and dermatomyositis. Array [online] 2013 December [viewed 10 December 2014] Available from: doi:10.1186/ar4263
  5. WEDDERBURN LUCY R., RIDER LISA G.. Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatment. Best Practice & Research Clinical Rheumatology [online] 2009 October, 23(5):665-678 [viewed 10 December 2014] Available from: doi:10.1016/j.berh.2009.07.007
  6. HUBER ADAM M., GIANNINI EDWARD H., BOWYER SUZANNE L., KIM SUSAN, LANG BIANCA, LINDSLEY CAROL B., PACHMAN LAUREN M., PILKINGTON CLARISSA, REED ANN M., RENNEBOHM ROBERT M., RIDER LISA G., WALLACE CAROL A., FELDMAN BRIAN M.. Protocols for the initial treatment of moderately severe Juvenile dermatomyositis: Results of a children's Arthritis and Rheumatology Research Alliance consensus conference. Arthritis Care Res [online] 2010 December:NA-NA [viewed 10 December 2014] Available from: doi:10.1002/acr.20071
  7. JOSHI DEEPAK, MAHMOOD RIZWAN, WILLIAMS PETER, KITCHEN PAUL. Dysphagia secondary to dermatomyositis treated successfully with intravenous immunoglobulin: a case report. Array [online] 2008 December [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-12
  8. CASTRO C., GOURLEY M.. Diagnosis and treatment of inflammatory myopathy: issues and management. Therapeutic Advances in Musculoskeletal Disease [online] December, 4(2):111-120 [viewed 10 December 2014] Available from: doi:10.1177/1759720X11425092

Management - Specific Treatments

Fact Explanation
Drug management for the muscle disease Mainstay of drug management for the muscle disease is corticosteroids, usually prednisolone. It should be slowly tapered once started to prevent the relapses. Occurrence of side effects should be monitored; peptic ulcers, osteoporosis, cushing's syndrome, diabetes mellitus etc and treated accordingly. Steroid sparing drugs also can be used when the steroid induced side effects are significant. Those steroid sparing drugs are Methotrexate, Azathioprine, Cyclophosphamide, Cyclosporine, Mycophenolate mofetil, Leflunomide and Chlorambucil. Other than that monthly high doses of IV immunoglobulin is beneficial in resistant cases. Monoclonal antibodies like rituximab are also shown to have benefits. [1][2][3][4][5]
Drug management for the skin disease The treatment of dermatological lesion is often difficult. Anti malarials such as hydroxychloroquine and chloroquine are shown to be beneficial. When those are contraindicated methotrexate can be used. Other than that IV immunoglobulin, Sirolimus, dapsone and rituximab also has benefit on skin lesions. [2][6][7]
Management of calcinosis Early treatment of myositis will reduce the complication, calcinosis. Calcium channel blockers such as diltiazem has place in gradual resolution of the calsinosis. Established calcinotic nodules can be removed surgically.[8]
Management of arrhythmia and cardiac failure Anti arrythmetics according to the type of arrhythmia has to be tried. For the cardiac failure spironolactone, ACE inhibitors and beta blockers can be used.[9]
References
  1. ALBAYDA J., CHRISTOPHER-STINE L.. Novel approaches in the treatment of myositis and myopathies. Therapeutic Advances in Musculoskeletal Disease [online] December, 4(5):369-377 [viewed 10 December 2014] Available from: doi:10.1177/1759720X12447705
  2. MARTIN N., LI C. K., WEDDERBURN L. R.. Juvenile dermatomyositis: new insights and new treatment strategies. Therapeutic Advances in Musculoskeletal Disease [online] December, 4(1):41-50 [viewed 10 December 2014] Available from: doi:10.1177/1759720X11424460
  3. LEVY DEBORAH M., BINGHAM C. APRIL, KAHN PHILIP J., EICHENFIELD ANDREW H., IMUNDO LISA F.. Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids. The Journal of Pediatrics [online] 2010 February, 156(2):302-307 [viewed 10 December 2014] Available from: doi:10.1016/j.jpeds.2009.09.008
  4. SCHIOPU ELENA, PHILLIPS KRISTINE, MACDONALD PAUL M, CROFFORD LESLIE J, SOMERS EMILY C. Predictors of survival in a cohort of patients with polymyositis and dermatomyositis: effect of corticosteroids, methotrexate and azathioprine. Array [online] 2012 December [viewed 10 December 2014] Available from: doi:10.1186/ar3704
  5. ODDIS CHESTER V., REED ANN M., AGGARWAL ROHIT, RIDER LISA G., ASCHERMAN DANA P., LEVESQUE MARC C., BAROHN RICHARD J., FELDMAN BRIAN M., HARRIS-LOVE MICHAEL O., KOONTZ DIANE C., FERTIG NOREEN, KELLEY STEPHANIE S., PRYBER SHERRIE L., MILLER FREDERICK W., ROCKETTE HOWARD E.. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: A randomized, placebo-phase trial. Arthritis & Rheumatism [online] December, 65(2):314-324 [viewed 10 December 2014] Available from: doi:10.1002/art.37754
  6. KIM JI EUN, JEONG MYEONG GIL, LEE HA EUN, KO JOO YEON, RO YOUNG SUCK. Successful Treatment of Cutaneous Lesions of Dermatomyositis with Topical Pimecrolimus. Ann Dermatol [online] 2011 December [viewed 10 December 2014] Available from: doi:10.5021/ad.2011.23.3.348
  7. CORDEIRO A C. Treatment of inflammatory myopathies. Postgraduate Medical Journal [online] 2006 July, 82(969):417-424 [viewed 10 December 2014] Available from: doi:10.1136/pgmj.2005.038455
  8. RAO V., BOWMAN S.. Latest advances in connective tissue disorders. Therapeutic Advances in Musculoskeletal Disease [online] December, 5(4):234-249 [viewed 10 December 2014] Available from: doi:10.1177/1759720X13480280
  9. DHOBLE ABHIJEET, PUTTARAJAPPA CHETHAN, NEIBERG ALAN. Dermatomyositis and supraventricular tachycardia. Array [online] 2008 December [viewed 10 December 2014] Available from: doi:10.1186/1755-7682-1-25