Dermatomyositis Sine Myositis Research Articles

Dermatologic Manifestations of Systemic Diseases in Childhood

Dermatologic Manifestations of Systemic Diseases in Childhood

The significance of dermoscopy and trichoscopy in differentiation of erythroderma due to various dermatological disorders.

The diagnosis of a patient with erythroderma may be difficult and sometimes pose a challenge for both dermatologist and pathologist. The role of dermoscopy in this area seems to be poorly investigated. There are only a few reports, with limited number of patients, describing dermoscopic features in erythroderma of various origins. To the best of our knowledge, none of the previous studies had included trichoscopic examination. Analysis of dermoscopic and trichoscopic patterns in series of patients with erythroderma. We retrospectively analysed 28 adult patients who presented with erythroderma between May 2016 and August 2020. Demographic data, disease course and duration, previous treatment, as well as dermoscopic and trichoscopic features were analysed. There were 9 patients (32.1%) with the diagnosis of mycosis fungoides, 8 patients (28.5%) with atopic dermatitis, 3 patients (10.5%) with Sézary syndrome and 3 patients (10.5%) with pityriasis rubra pilaris. The others were diagnosed with allergic eczema (n=1; 3.6%), dermatomyositis sine myositis (n=1; 3.6%), psoriasis (n=1; 3.6%), actinic reticuloid (n=1; 3.6%) and crusted scabies (n=1; 3.6%). Characteristic dermoscopic/trichoscopic patterns have been observed in erythroderma due to crusted scabies, psoriasis, dermatomyositis sine myositis, Sézary syndrome and pityriasis rubra pilaris. Differentiation of mycosis fungoides and long-standing atopic dermatitis based on dermoscopy is difficult, as the overlap of vessel morphology, background colour and scale colour exists. Similarly, differentiation between AD and AE based on dermoscopy/trichoscopy seems to be impossible, and clinical background is crucial. Dermoscopy and trichoscopy seem to provide additional clues in the assessment of erythrodermic patient. Depending on the underlying cause, trichoscopy or dermoscopy may be more useful.

18512 The significance of dermoscopy and trichoscopy in differentiation of erythroderma due to various dermatologic disorders

The diagnosis of an underlying cause of erythroderma may be difficult and often pose a challenge for dermatologist. There are only few case reports describing dermoscopic features in erythroderma of various origin. None of the previous studies analyzed trichoscopic patterns in this group of patients. The studied group comprised 19 adult patients with erythroderma (12 female/7 male, overall age range 18-88 y) in whom demographic data, disease course and duration, previous treatment, as well as dermoscopic and trichoscopic features were analyzed. There were 5 patients with the diagnosis of atopic dermatitis (AD), 6 patients with mycosis fungoides (MF), and 3 patients with Sezary syndrome (SS). The others were diagnosed with: allergic eczema (AE; n = 1), dermatomyositis sine myositis (DsM; n = 1), psoriasis (Pso; n = 1), actinic reticuloid (AR; n = 1), and crusted scabies (CS; n = 1). White scale, white-pinkish structureless areas, and combination of dotted, linear irregular, and spermatozoon-like shape vessels were the most common structures observed in MF (each in 100%); brown structureless areas were present in 66.7%. In patients with AD white scale, white-pinkish structureless areas, and dotted vessels were present in 100%; linear irregular vessels were present in 80%. In SS the most common structures were: white scale, dotted vessels, linear irregular vessels (each in 100%); glomerular vessels and gray dots were present in 66.7%. Characteristic dermoscopic/trichoscopic patterns seem to be particularly useful in initial recognition of SS, DsM, CS, and Pso. Trichoscopy provides additional, not previously reported, clues that may be useful in initial clinical differentiation of erythroderma due to various dermatologic disorders.

Cutaneous dermatomyositis in the era of biologicals.

Dermatomyositis (DM) is a systemic inflammatory condition characterized by cutaneous and muscle findings, in addition to potential involvement of other organ systems. A distinct subtype of DM exists that is categorized by cutaneous findings with absent or minimal muscle involvement, referred to as clinically amyopathic dermatomyositis or dermatomyositis sine myositis. A variety of topical, immunosuppressive, and immunomodulatory therapies have been utilized to treat cutaneous DM. The advent of biological agents including tumor necrosis factor-α antagonists, intravenous immunoglobulin, rituximab, and others has allowed for the use of these agents with varying degrees of success for the treatment of cutaneous DM.

Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

Dermatomyositis (DM) is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

Anticyclic Citrullinated Peptide Antibodies as Markers of Erosive Arthritis in Antisynthetase Syndrome: Table 1.

To the Editor: We read with interest the recent letter by Nagashima, et al 1, who suggested that destructive/erosive arthropathy in anti-Jo-1-positive patients with dermatomyositis (DM) sine myositis is another disease subset, classifiable as DM sine myositis overlapping with rheumatoid arthritis (RA). Moreover, the authors state that anticyclic citrullinated peptide antibodies (anti-CCP) are useful for distinguishing subluxing from destructive arthropathy in this setting. We describe our experience on the topic. We have followed 12 anti-Jo-1-positive patients (8 women, 4 men, … Address correspondence to Dr. Cavagna; E-mail: lorenzo.cavagna{at}unipv.it

Lipodystrophy in association with adult-onset dermatomyositis sine myositis: A rare manifestation

Lipodystrophy in association with adult-onset dermatomyositis sine myositis: A rare manifestation

Destructive Arthropathy Associated with Dermatomyositis Sine Myositis Positive for Anti-Jo-1 and Anti-Cyclic Citrullinated Peptide Antibodies

To the Editor: Polymyositis (PM) associated with anti-synthetase antibodies is often accompanied by arthritis, but it is usually mild and self-limiting1. Subluxing arthropathy associated with anti-Jo-1 antibody (anti-Jo-1)-positive PM or dermatomyositis (DM) has been described as a distinct subset of PM/DM2,3. It is characteristically a deforming, predominantly nonerosive arthropathy associated with subluxation of the interphalangeal (IP) joints, especially those of the thumbs. We encountered 2 patients who had severe, deforming, destructive arthropathy associated with DM sine myositis and who were positive for anti-Jo-1 and anti-cyclic citrullinated peptide (anti-CCP) antibodies. Both patients fulfilled the American College of Rheumatology (ACR) criteria for rheumatoid arthritis (RA). ### Patient 1 In 1996, a 57-year-old Japanese woman was referred to our hospital with dyspnea on exertion. She had Gottron’s sign in her hands, elbows, and knees, but there was no weakness, arthralgia, or Raynaud’s phenomenon. Interstitial lung disease (ILD) was detected by chest radiography. Serum creatine kinase (CK) was normal, anti-Jo-1 was positive at 1:2, and rheumatoid factor (RF) and anti-SSA antibody were negative. She was … Address correspondence to Dr. Nagashima; E-mail: naga4ma{at}jichi.ac.jp

Myosittspesifikke autoantistoffer

Myositis-specific antibodies (MSA) are autoantibodies that are almost exclusively detected in idiopathic inflammatory myopathies (IIM). This article provides an overview of these autoantibodies and how they can be used clinically to identify subgroups of IIM. The article is based on a non-systematic literature review and our own experience. MSA can be detected in up to 50 % of patients with IIM. Patients with anti-synthetase antibodies have a constellation of clinical findings termed "the anti-synthetase syndrome", in which interstitial lung disease dominates the clinical picture. Anti-Mi2 antibodies is another myositis-specific antibody. Patients with anti-Mi2 antibodies often have classical dermatomyositis, while the anti-SRP antibody identifies patients with severe myopathy, poor response to treatment with corticosteroids and histological findings of muscle cell necrosis - often lacking inflammatory infiltrates. The newly detected anti-CADMp140 appears to be associated with amyopathic or hypomyopathic dermatomyositis, previously called dermatomyositis sine myositis. Anti-p155 antibodies are most often found in patients who also have cancer. Myositis-specific antibodies may be useful for identification of clinical subgroups of IIM and can thereby affect the choice of medical treatment.

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.

Cutaneous symptoms of dermatomyositis significantly impact patients' quality of life

Dermatomyositis affects visible skin and causes disease symptoms that can affect patients' quality of life (QOL). In all, 71 patients with dermatomyositis or dermatomyositis sine myositis completed two QOL measures (the Skindex-16 and the Dermatology Life Quality Index) and a visual analog scale for pruritus. Disease severity was assessed by Physician's Global Assessment. The mean Dermatology Life Quality Index score was 10.7 and the mean Skindex-16 score was 51.1. Itching contributed to impact on both the Dermatology Life Quality Index and Skindex-16. Females reported worse QOL. The effect of treatment on quality of life was not assessed in these analyses. QOL impairment in dermatomyositis is greater than in other skin conditions including psoriasis and atopic dermatitis. Pruritus is an important treatable factor that significantly impacts QOL for patients with dermatomyositis.

Transient Elevation of the Tumor Markers CA 15-3 and CASA as Markers of Interstitial Lung Disease Rather than Underlying Malignancy in Dermatomyositis Sine Myositis

A 37-year-old woman with severe interstitial lung disease associated with dermatomyositis sine myositis is reported. A thoracoscopic lung biopsy revealed organizing diffuse alveolar damage. Significantly elevated serum levels of the tumor markers CA 15-3 and CASA (cancer-associated serum antigen) were detected, but no evidence of an underlying malignancy (including breast and ovarian) was found on serial clinical and radiologic examinations. These levels gradually normalized as the interstitial lung disease responded to a combination of cyclophosphamide and corticosteroids. The use of the CA 15-3 and CASA assays to measure serum levels of the highly glycosylated, high-molecular-weight mucin MUC1 in interstitial lung disease has not been previously described. Clinicians should therefore be aware that elevation of these tumor markers may reflect the presence of interstitial lung disease rather than an underlying malignancy in patients with dermatomyositis, especially if the levels normalize after successful treatment of the lung disease.

Dermatomyositis sine myositis and antisynthetase syndrome

We describe a 66-year-old woman with cutaneous lesions typical of classic dermatomyositis, clinical evidence of antisynthetase syndrome (arthritis, Raynaud’s phenomenon, mechanic’s hands and interstitial lung disease with anti-Jo-1 autoantibody) and lack of muscle disease after a full muscle evaluation that included clinical, enzymatic, electromyographic, magnetic resonance imaging and histological studies. The patient did not develop myositis after 9 years of clinical disease. The association of dermatomyositis sine myositis with antisynthetase antibodies suggests that the characteristic skin lesions are closely linked with dermatomyositis on the basis of the similar clinical and serological features of both dermatomyositis sine myositis and classic dermatomyositis.

Idiopathic inflammatory muscle disease: clinical aspects.

Although much remains to be learned about the immune-mediated myositis syndromes, information generated from recent studies in a number of areas may assist physicians in patient management. Topics reviewed here include: data supporting the association of myositis with cancer and the appropriate evaluations for malignancy in a myositis patient; an approach to the assessment of patients with dermatomyositis sine myositis; the usefulness of the clinicopathological and serological classifications; a discussion of whether childhood and adult myositis are the same or different entities; a review of those prognostic factors to consider in the clinical management of myositis patients; current approaches and their limitations for assessing disease activity and damage. To improve our limited understanding of the myositis syndromes, national and international collaborations are needed to obtain the necessary numbers of subjects, given the rarity and heterogeneity of these disorders.

Paraneoplastic amyopathic dermatomyositis associated with breast cancer recurrence

Amyopathic dermatomyositis, also known as dermatomyositis sine myositis, describes patients with the typical cutaneous features of dermatomyositis but without evidence of inflammatory myopathy. There have been 3 reported cases of amyopathic dermatomyositis associated with breast cancer in patients with no prior malignancy. We describe the first case of paraneoplastic amyopathic dermatomyositis associated with breast cancer recurrence. (J Am Acad Dermatol 1999;41:874-5.)

Rheumatic syndromes: Clues to occult neoplasia

Objectives: Rheumatic disorders associated with cancer include a variety ofconditions, most of which have no features distinguishing them from idiopathic rheumatic disorders. It is generally held that an extensive search for occult malignancy in most rheumatic syndromes is not recommended unless accompanied by specific findings suggestive of malignancy. The objective of this review are to identify rheumatic syndromes associated with cancer, to call attention to features that may suggest the presence of a hidden cancer, and to examine the role to additional clinical and laboratory data as clues to the possible neoplastic cause of those syndromes. Methods: A MEDLINE search of the literature dealing with cancer-associated rheumatic syndromes was conducted. Results: Review of the literature identified significant progress in this area. First, the association of malignancy with certain rheumatic syndromes was convincingly established, such as asymmetric polyarthritis presenting in the elderly with an explosive onset, rheumatoid arthritis with monoclonal gammopathy, Sjögren's syndrome with monoclonality, hypertrophic osteoarthropathy, dermatomyositis, polymyalgia rheumatica with atypical features, Lambert-Eaton myasthenic syndrome, palmar fasciitis and arthritis, eosinophilic fasciitis poorly responsive to corticosteroid therapy, erythema nodosum lasting more than 6 months, and onset of Raynaud's phenomenon or cutaneous leukocytoclastic vasculitis after age 50 years. Second, the list of cancer-associated rheumatic syndromes was extended by including additional entities such as benign edematous polysynovitis, sacroiliitis, adult-onset Still's disease, dermatomyositis sine myositis, systemic sclerosis, Sweet's syndrome, osteomalacia, skeletal hyperostosis, antiphospholipid syndrome, and essential mixed cryoglobulinemia. Third, evidence was provided substantiating that certain longstanding rheumatic syndromes, in particular rheumatoid arthritis, Felty's syndrome, Sjögren's syndrome, dermatomyositis, systemic sclerosis, systemic lupus erythematosus, and temporal arteritis behave like “premalignant conditions.” Fourth, it was shown that the recognized tumor markers alpha-fetoprotein, prostate-specific antigen, CA-125, CA 19-9, and CA-3 have low sensitivity and specificity in screening for occult cancer in a population of rheumatic patients, whereas the presence of a monoclonal gammopathy in rheumatoid arthritis and the monoclonal antibody 17-109 in Sjögren's syndrome are reliable signs of malignant transformation. Conclusions: The presence of specific rheumatic syndromes and certain clinical and laboratory findings may justify a workup for hidden cancer. Studies of the epidemiology of the cancer-associated rheumatic syndromes and evaluation of the validity of aforementioned clues in prospectives studies are goals for future investigations.

Dermatomyositis: A dermatology-based case series

Background: Dermatomyositis is associated with significant morbidity and occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis. Objective: Our purpose was to review the clinical features and response to therapy of patients with dermatomyositis and compare these data with previous series of patients with dermatomyositis/polymyositis. Methods: Clinical characteristics of 65 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontrolled study of treatment with high-dose prednisone followed by slow tapering. Results: Clinical features were similar to those previously described; however, muscle strength at diagnosis was on average greater in patients in this series than in patients previously reported. Malignancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue disease was present in 19% of patients. Twelve patients had dermatomyositis sine myositis. Eighteen of 21 patients (85%) in the prednisone study group had resolution of myositis. Conclusion: Patients with dermatomyositis in this series had less active myositis at presentation, but were otherwise similar to patients with dermatomyositis/polymyositis previously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective. (J Am Acad Dermatol 1998;38:397-404.)

Dermatomyositis sine myositis in five cases

Background A small subset of patients with dermatomyositis develop the characteristic cutaneous manifestations without muscle involvement, the so-called dermatomyositis sine myositis or amyopathic dermatomyositis. Whether systemic treatment of skin disease can prevent the development of myositis remains controversial.Objective The purpose of this study was to evaluate the course of the disease in terms of the onset of muscle symptoms under systemic treatment with corticosteroids or antimalarials.Methods Five patients with dermatomyositis sine myositis were included in this study. All skin biopsy specimens had features consistent with dermatomyositis. Muscle enzymes, electromyograms and muscle biopsies did not reveal any abnormality.Results Corticosteroids were given in three patients and hydroxychloroquine in two. Both regimens proved to be effective in the treatment of skin symptoms. Four patients, three on corticosteroids and one on hydroxychloroquine, did not develop muscle disease 4–7 years after presentation. One patient on hydroxychloroquine showed laboratory evidence of myositis 6 years after initiation of treatment.Conclusion It seems that muscle disease may appear within long periods of time after the onset of the cutaneous manifestations. The term dermatomyositis sine myositis can be used as a provisional diagnosis. Our observations suggest that systemic treatment of skin disease with corticosteroids is successful and may alter the disease course. Hydroxychloroquine may be helpful in some cases.

Dermatomyositis sine myositis in five cases

AbstractBackground A small subset of patients with dermatomyositis develop the characteristic cutaneous manifestations without muscle involvement, the so‐called dermatomyositis sine myositis or amyopathic dermatomyositis. Whether systemic treatment of skin disease can prevent the development of myositis remains controversial.Objective The purpose of this study was to evaluate the development of the disease in terms of the onset of muscle symptoms under systemic treatment with corticosteroids or antimalarials.Methods Five patients with dermatomyositis sine myositis were included in this study. All skin biopsy specimens had features consistent with dermatomyositis. Muscle enzymes, electromyograms and muscle biopsies did not reveal any abnormality.Results Corticosteroids were given in three patients and hydroxychloroquine in two. Both regimens proved to be effective in the treatment of skin symptoms. Four patients, three on corticosteroids and one on hydroxychloroquine, did not develop muscle disease 4–7 years after presentation. One patient on hydroxychloroquine showed laboratory evidence of myositis 6 years after initiation of treatment.Conclusion It seems that muscle disease may appear within long periods of time after the onset of the cutaneous manifestations. The term dermatomyositis sine myositis can be used as a provisional diagnosis. Our observations suggest that systemic treatment of skin disease with corticosteroids is successful and may alter the disease course. Hydroxychloroquine may be helpful in some cases.

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium‐201 scanning

AbstractBackground It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis.Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes.Methods In this single blind study we used thallium‐201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined.Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported.Conclusions Non‐invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.