Cutaneous Manifestations Of Dermatomyositis Research Articles

Muscle biopsy practices in the evaluation of idiopathic inflammatory myopathies: An international survey of expert clinicians

BackgroundMuscle biopsy is an important test in the evaluation of individuals with suspected myopathy, including those with suspected idiopathic inflammatory myopathy (IIM). Various approaches, including open surgical biopsy, needle biopsy and conchotome forceps, have been reported. However the real-world utilisation of these approaches remains unclear. There are no established guidelines for the use of muscle biopsy, or selection of biopsy technique, in investigating IIM and international practices are not well-documented. This study describes current approaches to muscle biopsy amongst clinicians with expertise in IIM. MethodsA survey regarding muscle biopsy practices was disseminated among members of the International Myositis Assessment and Clinical Studies (IMACS) group. Data were analysed using descriptive statistics. ResultsOne-hundred and sixteen clinicians completed the survey, primarily rheumatologists. Open surgical biopsy was the most commonly employed technique (74.5 %), followed by needle (11.3 %) and conchotome (9.4 %) approaches. Clinical examination was the most common method of muscle selection, with 85.2 % of respondents reporting they ‘always or almost always’ relied on it. MRI and electromyography were also frequently utilised for muscle selection (51.9 %, 45.4 % respectively). There was variability in the perceived utility of muscle biopsy in certain clinical contexts, such as presence of myositis specific antibodies or cutaneous manifestations of dermatomyositis. While respondents generally reported low complication rates following muscle biopsy, non-diagnostic histopathology was commonly reported, regardless of procedural approach. ConclusionClinicians managing IIM report muscle biopsy to be well tolerated however, non-diagnostic results are common. Substantial heterogeneity regarding perceived indications for biopsy, procedural approaches, and muscle selection strategies were observed within this expert group. Future research is needed to establish best practice and determine the role of muscle biopsy in the context of continued advancements in serological profiling of IIM.

Prevalence of cutaneous manifestations and myositis-specific antibodies in COVID-19 patients and Anti-PL7 antibodies association with pulmonary radiological severity: A retrospective study.

Dermatomyositis (DM) is an idiopathic immune-mediated myopathy, and may involve many organs, including muscles, skin and lungs. Myositis-specific autoantibodies (MSAs) are a useful aid in diagnosis DM and identifying its clinical subtype. During the COVID-19 pandemic, several studies found clinical similarities regarding lung involvement in both COVID-19 and DM. Such similarities have prompted speculation of a common pathogenetic mechanism. Indeed, viral infections are well-known triggers of autoimmune diseases. This prompted us to investigate whether circulating MSAs could be markers of the severity of lung involvement and of clinical outcome in COVID-19 patients. Moreover, we investigated the presence of cutaneous signs of DM in COVID-19 patients. We conducted a retrospective cohort study on 178 hospitalized patients affected by COVID-19. The diagnosis was confirmed by naso-pharyngeal swab positivity for SARS-CoV-2. The severity of lung involvement was assessed by assigning to each patient a radiological score ranging from 1 to 4, based on chest imaging (chest X-rays or CT scans). Serum samples were tested for MSAs. Anti-PL-7 antibodies were detected in 10.1% of patients and were found to be associated with an increased risk of severe pulmonary involvement (p = 0.019) and a worse prognosis in COVID-19 patients. Cutaneous lesions were observed in 26.4% of patients. However, none were cutaneous manifestations of DM. The detection of anti-PL7 antibodies might predict severe pulmonary involvement and a worse prognosis in COVID-19 patients.

Increased Epidermal Nerve Growth Factor without Small-Fiber Neuropathy in Dermatomyositis.

Small-fiber neuropathy (SFN) is suggested to be involved in the pathogenesis of some types of autoimmune connective tissue diseases. SFN with a reduction in epidermal nerve fibers might affect sensory fibers and cause neuropathic symptoms, such as pruritus and pain, which are common in both dermatomyositis (DM) and cutaneous lupus erythematosus (CLE). Nerve growth factor (NGF) has been recognized as important in nociception by regulating epidermal nerve fiber density and sensitizing the peripheral nervous system. The present study aimed to investigate whether SFN was associated with the cutaneous manifestations of DM and CLE. We also investigated the relationship between SFN and axon guidance molecules, such as NGF, amphiregulin (AREG), and semaphorin (Sema3A) in DM and CLE. To explore the molecular signaling, interleukin (IL)-18 and IL-31, which have been implicated in the cutaneous manifestation and neuropathic symptoms in DM, were examined in keratinocytes. Our results revealed that intraepidermal nerve fiber density (IENFD) was unchanged in patients with DM, but significantly reduced in IENFD in patients with CLE compared with healthy control. Increased epidermal expression of NGF and decreased expression of Sema3A were demonstrated in patients with DM. Furthermore, IL-18 and IL-31 both induced the production of NGF from keratinocytes. Taken together, IL-18 and IL-31 mediated epidermal NGF expression might contribute to the cutaneous neuropathic symptoms in DM, while SFN might be important for CLE.

Dermoscopy and Trichoscopy in Dermatomyositis-A Cross-Sectional Study.

Background: (Video)dermoscopy is a non-invasive diagnostic technique that has a well-established role in dermatooncology. In recent years, this method has also been increasingly used in the assessment of inflammatory dermatoses. So far, little is known about the (video)dermoscopic features of dermatomyositis (DM). Methods: Consecutive patients with DM were included in the study and videodermoscopic assessments of the nailfolds, scalp, and active skin lesions were performed. Results: Fifteen patients with DM (10 women and 5 men) were included. Capillaroscopy showed elongated capillaries (90.9%), avascular areas (81.8%), disorganized vessel architecture (81.8%), tortuous capillaries (72.7%), dilated capillaries (72.7%), and hemorrhages (72.7%). The trichoscopic findings included linear branched vessels (80.0%), linear vessels (60.0%), linear curved vessels (53.3%), perifollicular pigmentation (40.0%), perifollicular erythema (33.3%), scaling (20.0%), white (20.0%) or yellow (20%) interfollicular scales, and white (20.0%) or pinkish (13.3%) structureless areas. Polymorphic vessels of an unspecific distribution and white or pink structureless areas were frequently observed under dermoscopy in cutaneous manifestations of DM, including Gottron’s papules and Gottron’s sign. Conclusions: Dermoscopy of the nailfolds (capillaroscopy), scalp (tricoscopy), and active cutaneous lesions may be of value in the preliminary diagnosis of DM.

Hyperbaric oxygen therapy in the adjuvant treatment of cutaneous manifestations in dermatomyositis: a case report

Cutaneous manifestations of dermatomyositis are unusual and difficult to treat. This study aimed to report a case of cutaneous manifestations of dermatomyositis treated with hyperbaric oxygen. We present a case of dermatomyositis in a 44-year-old female with pain ulcers in her left leg for 17 months, refractory to an exclusive clinical treatment, who underwent a hyperbaric oxygen therapy (HBOT) breathing O2 100%, 90 minute sessions, six days a week, at 2.4 ATA. HBOT therapy proved to be highly efficacious in wound healing in this case and HBOT should be considered as a treatment in the assistance given to such patients.

Juvenile Onset Clinically Amyopathic Dermatomyositis Presenting as Calcinosis Cutis

Dermatomyositis (DM) is an autoimmune inflammatory disorder characterized by involvement of muscles and skin. Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. Rarely, DM presents with cutaneous features of the disease without muscle involvement for a period of more than six months. Such cases are classified into a category of clinically amyopathic dermatomyositis (CADM) which includes amyopathic DM and hypomyopathic DM. We present a case of a 14-year-old child who presented with calcinosis cutis and cutaneous findings suggestive of the disease without muscle involvement. The child was diagnosed as a case of juvenile onset CADM.J Enam Med Col 2018; 8(2): 101-104

Dermatomyositis: Current concepts

Dermatomyositis (DM) is a multifactorial chronic autoimmune disorder with characteristic skin changes and involvement of different organ systems, including the muscles, blood vessels, joints, esophagus, and lungs. In terms of epidemiology, DM affects both children and adults. It is most often observed beyond the age of 40, but there is also a peak of incidence between 5 and 12 years of age. The current paradigm describing the pathophysiology of DM is an autoimmune attack on affected organs that is triggered by environmental factors such as UV exposure, drugs, infection, and lifestyle decisions in genetically susceptible individuals. Importantly, DM is also regarded as a paraneoplastic phenomenon, as cancer may precede, occur concurrently with, or follow the development of the clinical signs of DM. The cutaneous manifestations of DM can be categorized as pathognomonic, characteristic, compatible, less common, rare, recent, and nonspecific. The treatment of DM is a difficult task due to its rarity, its multiple phenotypes, and the fact that the disease may affect multiple organs and is commonly treatment-refractory. The lack of randomized, controlled intervention trials and, until recently, the insufficiency of validated, clinically meaningful outcome instruments in part contribute to the lack of approved treatments.

Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15years of age and one in adulthood between 40 and 60years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking. The etiopathogenesis is still unclear, but a range of factors such as genetic predisposition, environment triggers, and immune- and non-immune-mediated mechanisms play a role in the development of the disorder. Some dermatological findings and new discoveries on immunological mechanisms in DM, including the association of certain autoantibodies with cancer, can be helpful in clinical practice. Therapy should consider both skin and muscle or internal organ involvement. Photoprotection, including the use of antimalarial drugs, plays an important role in the therapeutic approach of skin involvement. Treatment of muscle and internal organ involvement is based on systemic corticosteroids, alone or in association with disease-modifying antirheumatic drugs. Intravenous immunoglobulins have been demonstrated to be effective as second-line therapy. Rituximab is an emerging treatment for difficult-to-treat DM, both in adults and children. In this review article, we discuss DM subforms, focusing on their dermatological manifestations that can be classified as pathognomonic, characteristic, compatible, less common, rare, recently-described, and non-specific skin lesions.

Bridge to diagnosis: The use of extracorporeal membrane oxygenation in a child with interstitial lung disease secondary to clinically amyopathic dermatomyositis

Abstract Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis (DM), is characterized as having the classic cutaneous manifestations of DM but with little or no evidence of myositis. The presence of anti-melanoma differentiation associated gene 5 (anti-MDA-5) autoantibodies, in the setting of CADM, is associated with rapidly progressive interstitial lung disease (RPILD). The use of extracorporeal membrane oxygenation (ECMO) in patients with RPILD has been well described in adults, but not in children. Herein we present a 14-year old patient with interstitial lung disease, initially of unknown etiology, whom we supported with ECMO in the acute phase of her illness for nearly two months during which the diagnosis of anti-MDA-5 dermatomyositis was established.

Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features-a Comprehensive Review.

Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product(MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

Cutaneous manifestations of dermatomyositis in male patient: a rare report

Introduction: Dermatomyositis is an idiopathic inflammatory myopathy. It is a systemic disorder that most frequently affects the skin and muscles, but may also affect the joints, the esophagus, the lungs, and, less commonly, the heart. Case report: It is presented with characteristic cutaneous findings like skin rash as well as progressive symmetrical proximal muscle weakness. Its prevalence rate is approximately one per 100,000 in the general population with a female to male predominance of about 2:1. Conclusion: We report a male patient with the classical features of dermatomyositis in whom cutaneous changes preceded muscle weakness.

Dermatomiositis asociada a cáncer de mama. Síndrome paraneoplásico infrecuente

Dermatomyositis is a rare paraneoplastic syndrome associated with several malignant tumors. The association with breast cancer is much less frequent. We report a 50-year-old woman who presented with dermatomyositis and was diagnosed with breast cancer. Surgical treatment of breast cancer improved the symptoms of muscular weakness and the cutaneous manifestations of dermatomyositis.

Cutaneous Manifestations of Dermatomyositis and Their Management

Dermatomyositis is a condition with pathognomonic and characteristic cutaneous lesions. This article describes the skin manifestations observed in patients with dermatomyositis, their differential diagnosis, their relationship to internal disease (particularly malignancy), and their management.

Amyopathic dermatomyositis or dermatomyositis-like skin disease: retrospective review of 16 cases with amyopathic dermatomyositis

Amyopathic dermatomyositis (ADM) is characterized by the presence of dermatomyositis (DM) for 6 months or more in individuals who have normal muscle enzymes and no clinically significant muscle weakness. The aim of the study was to investigate the initial laboratory data, clinical manifestations, complications, and clinical outcomes of patients with the diagnosis of ADM. We reported 16 cases with the cutaneous findings of dermatomyositis without clinical or laboratory evidence of muscle disease for at least 2 years after onset of the skin manifestations in the Department of Dermatology and Rheumatology at Shanghai Ruijin Hospital between 1998 and 2004. All patients had Gottron's papules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. Follow-up of 1 to 10 years after diagnosis found muscle weakness in three patients (18.75%) within 5 years of diagnosis. One patient (6.15%) was rediagnosed as chronic cutaneous lupus erythematosus (CCLE). Four patients (25%) had associated malignancies. Twelve patients (75%) had radiographic evidence indicative of interstitial fibrosis irrespective of respiratory symptoms. Patients with ADM appear to be at risk for developing the same potentially fatal disease complications as those patients with DM (e.g., interstitial lung disease and internal malignancy). These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic for DM and we propose the term dermatomyositis-like skin disease as a better designation than amyopathic dermatomyositis to describe this distinctive subset of cutaneous symptoms. Dermatomyositis-like skin disease is a complex syndrome, which includes the characteristic cutaneous eruption of dermatomyositis without clinical evidence of muscle disease. Our findings suggest that patients diagnosed with this syndrome are at risk for fatal interstitial lung disease, malignancy, and/or delayed onset of DM or CCLE. Cautious systematic clinical trials should be considered for this group of patients.

A systematic review of juvenile-onset clinically amyopathic dermatomyositis

Dermatomyositis (DM) presenting during childhood or adolescence classically encompasses hallmark cutaneous changes, proximal muscle weakness, and laboratory evidence of myositis. When cutaneous manifestations of DM are present without muscle weakness for > 6 months, the term 'clinically amyopathic DM' applies (syn. DM sine myositis). To review the clinical and epidemiological features of published cases of juvenile-onset clinically amyopathic DM, with comparison with adult-onset clinically amyopathic DM and juvenile-onset classical DM. Systematic review of the published literature. We identified 68 cases of juvenile-onset clinically amyopathic DM published during 1963-2006. The disease in 18 of 68 (26%) patients subsequently evolved to classical DM. Overall, the mean age at diagnosis was 10.8 years (range 2-17) with nearly equal male/female ratio and mean follow-up of 3.9 years. Among cases with diagnostic testing, 10 of 19 had a positive antinuclear antibody titre, two of nine had elevated erythrocyte sedimentation rate and two of 51 had elevated creatine kinase (CK). Of patients with normal CK, three of 22 had abnormal electromyography, one of 19 had abnormal muscle biopsy, and one of nine had abnormal magnetic resonance imaging. Calcinosis was reported in three of 68. No cases of severe vasculopathy (resulting in ulceration), interstitial lung disease or internal malignancy were reported. This review suggests a good prognosis for children with clinically amyopathic DM. A minority of patients with negative muscle enzymes had positive ancillary testing for myositis, and these patients rarely developed muscle weakness. Predictive factors for progression to classical DM were not identified. Symptomatic treatment of cutaneous involvement and close clinical monitoring may be an alternative to aggressive immunosuppression.

Peripheral T Cell Lymphoma Presenting as Dermatomyositis-like Eruption

Background: There are several conditions reported to mimic the cutaneous manifestations of dermatomyositis, including lymphoproliferative disorders. Objective: This case report presents an unusual case of peripheral T cell lymphoma mimicking dermatomyositis and discusses the clinical and pathologic features that distinguish it from dermatomyositis. Methods and Results: A 62-year-old woman presented with a two-month history of a progressive painful cutaneous eruption and interstitial infiltrates on chest x-ray. Skin biopsy revealed peripheral T cell lymphoma. The diagnosis was confirmed by lung biopsy. Conclusions: Although rare, a lymphoproliferative disorder must be included in the differential diagnosis of a cutaneous dermatomyositis-like eruption.

Cutaneous involvement of dermatomyositis can respond to Dapsone therapy

Dapsone (4,4-diaminodiphenylsulfone) is a sulfone antibiotic used in the treatment of leprosy, but dermatologists more commonly utilize its anti-inflammatory properties particularly directed against leukocytes to treat various bullous disorders, erythema nodosum, pyoderma gangrenosum, Sweet's syndrome, cutaneous vasculitis, and cutaneous forms of lupus erythematosus. The cutaneous manifestations of dermatomyositis are often resistant to antimalarial and immunosuppressive therapies. Two patients with cutaneous dermatomyositis unresponsive to combination therapy with prednisone, hydroxychloroquine, quinacrine, and immunosuppressive medications had Dapsone added to their therapy. Both patients showed rapid improvement with the addition of Dapsone. Each had an exacerbation of their cutaneous dermatomyositis on Dapsone with withdrawal and subsequent improvement when the Dapsone was reinstituted. Dapsone therapy for cutaneous dermatomyositis may have a wider role in treatment for these patients refractory to prednisone and antimalarial therapy.

The use of chloroquine and hydroxychloroquine for non-infectious conditions other than rheumatoid arthritis or lupus: a critical review

Chloroquine and hydrocychloroquine have been evaluated in 30 noninfectious disorders and conditions other than rheumatoid arthritis or lupus erythematosus; 12 of these have been subjected to well-designed controlled trials. It is concluded that chloroquines are safe and effective first line therapies for selected patients with porphyria cutanea tarda, cutaneous sarcoidosis, cutaneous manifestations of dermatomyositis, hyperlipidemias and thromboembolic prophylaxis for patients with antiphospholipid antibodies. Published experience with these and other diseases or syndromes are critically reviewed.

Amyopathic dermatomyositis: a review.

Jim Gilliam's research interests throughout his career were forced upon better defining the relationships that exist between the cutaneous and systemic manifestations of the rheumatic diseases. Although the majority of his time was spent studying such relationships in lupus erythematosus patients, he was also intensely interested in dermatomyositis (DM) in this regard as well. He was particularly intrigued with the dissociation of the cutaneous and muscular manifestations of this disorder that occasionally occurs. The term "dermatomyositis siné myositis" has been used in the past to describe patients who present with only the cutaneous manifestations of DM; however, very little published data is available from systematic examinations of such patients. For several reasons, we have preferred the term "amyopathic dermatomyositis" to describe that rare patient who for long periods of time suffers from the classical skin lesions of DM as the only clinically significant manifestation of their disease. In this presentation, we review our own personal experience with a group of six such patients and compare and contrast it to that of other workers who have dealt with this subject over the past two decades.