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Inflammatory Myopathies with Cutaneous Involvement

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Inflammatory Myopathies with Cutaneous Involvement DOI: 10.1515/folmed-2017-0003 REVIEW Infl ammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy Lyubomir A. Dourmishev Department of Dermatology and Venereology, Medical University of Sofi a, Sofi a, Bulgaria Correspondence: The group of idiopathic infl ammatory myopathies (IIM) include various disorders Lyubomir A. Dourmishev, Depart- of skeletal muscles with or without skin involvement. The most common types are ment of Dermatology and Venere- dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and nec- ology, Medical University of Sofi a, rotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various 1 St Georgi Sofi iski St., clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, 1431 Sofi a, Bulgaria E-mail: [email protected] scleromyositis, overlap or anti-synthetase syndromes, etc. Tel: +3592 9230438 Recently, numerous new antibodies defi ning the characteristic clinical phenotype Received: 08 March 2016 have been described as anti-MDA5 antibodies associated with interstitial lung Accepted: 19 July 2016 disease and amyopathic dermatomyositis or anti-TIF1γ antibodies as markers for Published Online: 22 Nov 2016 paraneoplastic dermatomyositis. Moreover, new clinical entities as drug-induced Published: 27 March 2017 dermatomyositis are presumed, since some medications may induce, or trigger infl ammatory myopathies. Key words: idiopathic infl am- matory myopathies, cutaneous Knowledge of the complex methods and techniques required to diagnose the involvement, clinical variants, disease is of great importance in clinical practice. The variety of clinical variants diagnosis, therapy needs diagnosis because of the diff ering prognosis and therapeutic modalities. Citation: Dourmishev LA. Infl am- matory myopathies with cutane- ous involvement: from diagnosis to therapy. Folia Medica 2017;59(1):7-13. doi: 10.1515/folmed-2017-0003 INTRODUCTION some medicaments may induce or trigger clinical 5 The group of idiopathic infl ammatory myopathies variants of myositis. This heterogeneity presumes (IIMs) include various disorders of skeletal muscles different pathogenesis and refl ects on clinical course 6 with or without skin involvement such as derma- and therapeutic response. tomyositis (DM), polymyositis (PM), inclusion In polymyositis (PM), patients present with a body myositis (IBM) and necrotizing autoimmune muscle syndrome, laboratory signs of skeletal muscle myopathy (NAM).1 Dermatomyositis is a rare idio- infl ammation but no skin lesions. In contrast to other pathic infl ammatory myopathy with a specifi c skin non-infl ammatory myopathies, PM patients have no syndrome. The disease itself expresses heterogene- family history of neuromuscular disease, exposure ity not only in clinical presentation, but also in the to myotoxic drugs or toxins, and clinical features 2 course and prognosis. Some of the patients may of endocrinopathy. have concomitant neoplastic disorders and features Inclusion body myositis is a rare infl ammatory of other connective tissue diseases or markers of au- myopathy which is prevalent in men. It presents toimmunity varying from absence of auto-antibodies with asymmetric muscle affection and atrophy of to very high titres of auto-antibodies.2 Various auto- distal muscles, lack of peripheral refl ex, rarely as- 7 antibodies found in dermatomyositis may distinguish sociates with malignancy and therapeutic resistance. certain clinical variants as anti-synthetase antibod- In IBM, muscle biopsy and electron microscopy ies in anti-synthetase syndrome (ASS), anti-TIF1γ show basophilic vacuoles of ectopic beta-amyloid in paraneoplastic dermatomyositis3 or anti-MDA5 and ubiquitin found in myofi brils. Familial cases of antibodies in amyopathic dermatomyositis4. Finally, IBM with VCP gene mutation and specifi c antibod- 7 Folia Medica I 2017 I Vol. 59 I No. 1 L. Dourmishev et al ies against 43-kDa muscle antigen in sera suggest tion of serum levels of skeletal-muscle enzymes; different aetiology and pathogenesis of IBM from 3) electro-myographic features of muscle affection; those of other IIMs.8 4) infl ammatory infi ltration, degeneration or atrophy Necrotizing autoimmune myopathy is character- in muscle biopsy; 5) presence of myositis specifi c ized with myositis, necrotic changes in muscle biopsy, auto-antibodies in sera; and 6) typical skin rash of highly elevated creatine kinase (CK) levels, and dermatomyositis. anti-HMGCR or other myositis specifi c antibodies Cutaneous lesions in DM are subdivided into (MSA) in sera, and is frequently associated with pathognomonic (highly specifi c and typical of dis- malignancy or treatment with statins and other drugs.9 order), characteristic and occasional.2,21 Pathogno- Dermatomyositis is a relatively rare disorder, with monic lesions are heliotrope erythema and edema incidence varying between 2 and 19 cases per 1 mil- of the eyelids, Gottron papules and Gottron sign. lion population.10,11 It affects women about twice as Heliotrope erythema is most frequently observed; frequently as it does men.11,12 The age distribution however, it is a less specifi c sign than Gottron’s shows a bimodal pattern with an initial peak at 10 papules. It usually is associated with periorbital years of age for juvenile DM and a second larger oedema and clinically presents as violaceous dusky peak between 40 and 60 years of age.11,12 macular erythema involving symmetrically the The aetiology of DM and PM remains unclear; eyelids, the upper cheeks and forehead.22 Gottron’s however, various exogenous factors as climate, papules express as bluish-red, slightly elevated vio- geographic latitude and sun exposure are found to laceous papules or plaques overlying the proximal be important in disease onset. An increasing rela- or distal dorsal interphalangeal or metacarpophalan- tive prevalence of DM in southern Europe (Greece geal joints, elbow, or knee joints.22 Characteristic and Italy) and higher incidence of PM in northern but less specifi c cutaneous manifestations include: European countries (Finland, Iceland, and Sweden) Shawl sign; nailfold telangiectasias; scalp scaly has been reported, presuming the ultraviolet irradia- disease and photosensitive poikiloderma. Rarely tion is an important triggering, aggravating factor observed skin lesions such as cutaneous vasculitis, for DM but not for PM.13 Epidemiological studies panniculitis, ulcerations, calcinosis, hands hyperkera- in the USA demonstrated an increased frequency tosis (mechanic’s hands), follicular hyperkeratosis, of adult and juvenile DM onset in spring and sum- centripetal linear or fl agellate erythema, Holster mer months.14 Various infectious agents as viruses, sign, erythroderma, and vesiculo-bullous lesions bacteria or protozoa (Toxoplasma gondii) were are compatible with DM.22 discussed in the past as the disease triggers.15,16 Clinical or minimal erythema dose tested pho- Other proposed factors as vaccines, immunoglobu- tosensitivity is observed in about half of patients lins against various viruses, as well as NSAIDs, with DM.23 Nailfold capillaroscopy features of the anti-neoplastic and anti-infectious drugs could be patients with dermatomyositis include giant capillar- responsible for triggering an autoimmune process.5 ies, microhemorrhages, capillary loss and avascular Cholesterol-lowering statins have been found to be areas that are barely distinguishable from systemic associated in some cases with the development of sclerosis.24 Histological features of skin biopsy in- necrotizing autoimmune myopathy.9 clude vacuolar (hydropic) degeneration of the basal Genetic factors are also found to be of major cell layer of the epidermis, necrotic keratinocytes, importance, as in other autoimmune diseases. HLA vascular dilatation and a superfi cial, perivascular DRB1*0301; DQA1*0501 have been determined to lymphocytic infi ltrate.25 be risk factors for all of the major clinical forms Muscles are almost always affected with my- of PM and DM.17 HLA-DRB1*03 is signifi cantly algia and symmetric progressive muscle weakness associated with anti-Jo-1-positive DM and PM.18 involving proximal muscle groups of extremities. Some TNF-308 A promoter gene polymorphisms Serum muscle enzymes as creatine kinase, aspartate have been found signifi cantly increased in both aminotransferase (AST), alanine aminotransferase juvenile and adult DM.19,20 (ALT), lactate dehydrogenase (LDH) and aldolase are usually elevated and mark the disease activity. DIAGNOSIS Muscle histology in DM reveals myofi ber necrosis, Diagnosis of IIMs is made following the criteria perifascicular atrophy, infl ammatory lymphocytic modified by Targoff et al.21 These include: 1) infi ltrate in the endomysium and perivascular in- symmetric proximal muscle weakness; 2) eleva- fl ammation in the perimysium.26 Histopathology 8 Folia Medica I 2017 I Vol. 59 I No. 1 Infl ammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy from muscle in PM shows myofi ber necrosis, an with neoplastic disorders.37 endomysial infl ammatory infi ltrate, perimysial and In 1916 Stretz introduced the association of perivascular infl ammation but no evidence of peri- dermatomyositis and cancer38 as paraneoplastic fascicular atrophy and no invasion of infl ammatory dermatomyositis (PDM). The frequency of the cells in normal muscle fi bres.26 PDM varies between 10 - 30% of dermatomyositis Lung involvement presents with interstitial pneu- patients.39-41 Malignancy can precede occurring si- monia, diffuse alveolitis or bronchiolitis obliterans
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