Clinical Features Of Dermatomyositis Research Articles

Clinical features, treatments and outcomes of calcinosis in adult patients with dermatomyositis: a single cohort study.

The occurrence of calcinosis cutis as a clinical feature of dermatomyositis in adult patients is not well understood. Cohort studies of adult patients with calcinosis are rare. We systematically describe the clinical features, treatments and outcomes of adult patients with calcinosis. We initially enrolled 627 adult DM patients. Of those enrolled, 35 (5.6%) were found to have calcinosis. We analysed the clinical features associated with calcinosis in this subgroup. The risk factors associated with calcinosis were analysed using the Poisson regression model. Multivariate analysis showed that a younger age at disease onset [odds ratio (OR) = 0.945, 95% CI 0.925, 0.966, P < 0.001], dysphagia (OR = 2.609, 95% CI 1.189, 5.728, P = 0.017), skin ulcer (OR = 5.705, 95% CI 3.041, 10.705, P < 0.001) and the presence of anti-nuclear matrix protein 2 antibody (OR = 5.917, 95% CI 2.754, 12.714, P < 0.001) were independently associated with calcinosis. In both the low- and high-dose prednisone treatment groups, no difference in treatment response was seen between the bisphosphonate treatment group and the group not receiving bisphosphonate treatment (P = 1.000 and P = 0.375, respectively). A follow-up study revealed that the mortality rate of the calcinosis group was 5.7%. Additionally, 60.6% of the cases had a chronic polycyclic disease course and 17.1% had mild complications secondary to calcinosis. Calcinosis is an uncommon, but significant clinical feature in adult patients with DM. Bisphosphonates were not found to effectively treat calcinosis, however, the overall health outcomes of adult DM patients with calcinosis were favourable.

Tracking changes in nailfold capillaries during dermatomyositis treatment

To the Editor: The clinical features of dermatomyositis are believed to be due in part to small vessel vasculopathy and perivascular inflammation, which leads to a reduction in capillary density with compensatory dilatation of the remaining capillaries. 1 Dalakas M.C. Inflammatory muscle diseases. N Engl J Med. 2015; 373: 393-394 Crossref PubMed Scopus (2) Google Scholar In the skin, a manifestation of this is easily measurable is changes in capillary nailfolds. Indeed, a retrospective study of 92 juvenile dermatomyositis patients showed that nailfold capillary density is a marker of not just skin damage but also muscle damage and, therefore, appears to reflect the degree of systemic blood vessel alterations. 2 Schmeling H. Stephens S. Goia C. et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford). 2011; 50: 885-893 Crossref PubMed Scopus (52) Google Scholar Another study of 60 patients with juvenile dermatomyositis showed that low nailfold capillary density is associated with impaired pulmonary function tests. 3 Barth Z. Schwartz T. Flatø B. et al. The association between nailfold capillary density and pulmonary and cardiac involvement in medium- to long-standing juvenile dermatomyositis. Arthritis Care Res (Hoboken). 2018; ([Epub ahead of print]) PubMed Google Scholar The question remains if these changes in adult dermatomyositis are permanent and represent a persistent measure of overall disease-induced damage, or if on therapy, they are reversible, suggesting they are more a measure of disease activity. Our objective was to assess if these capillary changes reverse with the use of immunosuppressant therapy and if the rate or extent of reversal varies between therapeutic options.

11. A comparative study of the clinical features of dermatomyositis in child and in adult patients where the Anti-Mi-2 antibody is present

11. A comparative study of the clinical features of dermatomyositis in child and in adult patients where the Anti-Mi-2 antibody is present

Dermatomyositis as initial presentation of Hodgkin's lymphoma: A case report and review of literature

The increased incident of malignancy is well established in patients with inflammatory myopathies and specifically in Dermatomyositis (DM). However, the association between DM and Hodgkin’s Disease (HD) is very unusual. We have recently had the opportunity of observing a patient who manifested the clinical features of DM and found to have HD after a detailed work up. You can submit your Manuscripts at: https://symbiosisonlinepublishing.com/submitManuscript.php

Anti-CADM-140/MDA5 antibody and clinical subsets of dermatomyositis

The clinical features of dermatomyositis (DM) have a close relationship with myositis-specific antibodies. Clinically amyopathic dermatomyositis (CADM) is a subgroup of DM, which manifests as characteristic skin symptoms compatible with DM, such as Gottron's sign and heliotrope rash with no or mild muscle symptoms. Sometimes, a life-threatening rapidly progressing interstitial lung disease can complicate CADM. In recent years, anti-CADM-140/MDA5 antibodies have been observed in serum obtained from patients with CADM. Thus, measurement of antiCADM-140/MDA5 antibodies is useful for the diagnosis and prediction of prognosis of patients with CADM. Rec.4/8/2013, Acc.5/10/2013, pp223-227

Dermatomyositis: a contemporary review for oral health care providers

Dermatomyositis (DM) is an autoimmune muscle disorder characterized by skin rashes and progressive muscle weakness. This disease is a subset of the idiopathic inflammatory myopathies (IIM) and affects both children and adults. Increased incidence of malignancy has been observed in adult DM, making early diagnosis, treatment, and monitoring crucial. In the past, limitations of these diagnostic criteria and classifications often made it difficult to identify the disease in the early stages. However, in recent years a new understanding of the pathogenesis and clinical features of DM has developed, which enables clinicians to distinguish DM from other autoimmune disorders and other subsets of IIM. Many signs and symptoms of DM involve the orofacial regions. This paper reviews and updates the disease process, orofacial manifestations, and dental considerations for patients with DM. Understanding new knowledge of DM helps oral health care providers coordinate care for patients with this disease.

Sweet-like Dermatosis in 2 Patients With Clinical Features of Dermatomyositis and Underlying Autoimmune Disease

The neutrophilic dermatoses comprise a group of cutaneous disorders that are characterized histopathologically by infiltration of the dermis with mature neutrophils with or without vessel wall destruction. Neutrophilic dermatoses have been reported in association with a variety of autoimmune diseases, most recently as a manifestation of lupus erythematosus. We describe 2 patients with photodistributed violaceous plaques: one with associated heliotrope rash and malar erythema, and the other with scalp involvement and Gottron-like papules. In each case, the biopsy specimen revealed changes compatible with a neutrophilic dermatosis as opposed to an interface dermatitis. The first patient also had a history of Graves disease and primary biliary cirrhosis, while second patient had Wegener granulomatosis. The 2 patients responded to therapy with oral dapsone and prednisone, respectively. The atypical presentation of neutrophilic dermatosis in 2 patients with clinical features of dermatomyositis and intercurrent autoimmune-mediated illnesses may suggest an expansion in the clinical spectrum of parainflammatory neutrophilic dermatoses. The finding of a neutrophilic dermatosis in a biopsy specimen from a patient without a classic clinical presentation should invoke a thoughtful search for underlying immune complex-mediated systemic disease.

A Case of Lobular Panniculitis in Dermatomyositis Patient

Panniculitis is an inflammation of subcutaneous fat tissue, and found in variety of clinical setting, like erythema nodosum, lupus or other connective tissue diseases. Panniculitis, as a clinical feature of dermatomyositis, is rare and should be included in one of the sign to be differentiated. We present a 77-year-old female dermatomyositis patient who showed a clinically and histologically evident lobular panniculitis, and successfully treated with oral prednisolone.

The clinical features of dermatomyositis in a South Australian population

Aim: To review the clinical features of dermatomyositis (DM) in a South Australian population. Methods: Retrospective review of medical records of patients with biopsy-proven DM in South Australia from 1990 to 2005. Results: There were 21 cases of biopsy-proven DM in SA (62% F, mean age 49.7 ± 18.4 years) and clinical details were available in 20 of these. Malignancy was identified in 9/20 patients; in five this followed the diagnosis of DM, with three malignancies seen within 3 months of disease onset. Three patients had a clearly defined immune insult prior to the diagnosis of DM; one patient had Mycoplasma pneumoniae infection 23 days prior to DM, two had pneumococcal and influenza vaccinations 5 and 14 days prior to the onset of DM, respectively. Two of three patients with anti-Jo-1 antibody experienced thromboembolism within 2 months of DM onset and three patients had interstitial lung disease (2 with anti-Jo-1 antibody). Creatine kinase (CK) was elevated in 15/20 cases and showed strong correlation with transaminases, and notably not with traditional inflammatory markers. Conclusions: This retrospective review of patients with biopsy-proven DM suggests a role for infection/vaccination in triggering disease onset. A particularly strong association with malignancy was observed and it is suggested that DM may predispose to thrombosis. Transaminases, in addition to CK may be used to monitor disease activity, and traditional inflammatory markers have little role in this.

Primary lung cancer revealed by dermatomyositis and detected by whole-body scintigraphy with 99mTc-HMDP

Dermatomyositis, an inflammatory muscle disease probably related to dysimmunity, is associated with character skin eruptions. Dermatomyositis is often associated with cancer (15 to 40% of cases depending on the series). All histological types and all cancer localizations observed in the general population can be associated with dermatomyositis. We report the case of a patient with small-cell lung cancer revealed by dermatomyositis appearing by a diffuse and intense uptake of 99mTc-HMDP in the whole-body scintigraphy.There have been few descriptions of this association in the literature. Certain clinical features of dermatomyositis would be predictive of its paraneoplastic nature. Prognosis is very poor. Treatment is basically dictated by the underlying neoplasia.

Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis

Multicentric reticulohistiocytosis (MRH) is a rare disorder with proliferating histiocytes that develop into multinucleated giant cells with “ground-glass” cytoplasm. The disease presents with a rapidly destructive, sometimes permanently debilitating, polyarthritis and a papulonodular eruption, generally of the face and hands. We present 3 cases of MRH in which the initial clinical diagnosis was thought to be dermatomyositis (DM). The cutaneous findings in these cases included an erythematous, predominantly photodistributed macular and papular eruption clinically consistent with DM. However, skin biopsy specimens revealed the diagnosis of MRH. This previously unreported clinical similarity between MRH and DM is significant because the treatment options and future complications vary greatly between the 2 diseases. Thus, it is important to consider the possibility of multicentric reticulohistiocytosis when evaluating a patient with the clinical diagnosis of dermatomyositis. (J Am Acad Dermatol 2003;48:S11-4.)

The red face: Dermatomyositis

Dermatomyositis (DM) is a disease that manifests as an inflammatory myopathy typically producing a symmetric proximal muscle weakness and a characteristic cutaneous eruption. 1–3 This disorder is closely related to polymyositis (PM), which has all the clinical features of DM without the presence of skin disease. Both of these disorders are of unknown etiology, but pathogenetic mechanisms are believed to involve immunologically mediated muscle damage. 4 Both DM and PM may occur in the presence of other collagen vascular diseases such as lupus erythematosus, scleroderma, Sjögren's syndrome, rheumatoid arthritis, and various vasculitides. Dermatomyositis seems to be characterized by an increased frequency of internal malignancy, whereas malignancy association in patients with PM is less clearly established. Recent data suggest that PM, as well as DM, occurs in children. Both DM and PM are associated with morbidity and mortality and, therefore, prompt and aggressive therapy is necessary. In this review we emphasize practical clinical aspects of DM.