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Dermatomyositis is a multisystem autoimmune connective tissue disease that can manifest with proximal muscle weakness, interstitial lung disease, and characteristic skin findings such as erythema and papules over the dorsal joints of the hand, periungual changes, heliotrope rash, and pruritic erythematous inflammation of the chest, back, arms, legs, and scalp. Erythromelalgia is a clinical diagnosis characterized by swelling, erythema, and burning pain, typically of the extremities and often triggered by heat or exertion. It can be classified as primary, due to genetic mutations, or secondary, associated with myeloproliferative, vascular, musculoskeletal, or neurologic disease.1 There have been reports of biopsies of erythromelalgia having some features of connective tissue disease.2,3 There are also reports of erythromelalgia associated with systemic lupus erythematosus and rheumatoid arthritis, two autoimmune connective tissue diseases.4 Here, we present two cases of erythromelalgia associated with dermatomyositis, which, to our knowledge, has not been reported in the literature to date. Case 1 An 81-year-old woman with a history of treated breast cancer presented to the dermatology clinic for evaluation of swelling and discoloration of her bilateral hands and feet of approximately 10 years' duration associated with heat intolerance. She had been seen by numerous vascular and lymphedema specialists over the years with the ultimate conclusion that her swelling was not associated with a vascular etiology. The patient noticed improvement of her symptoms when she was exposed to air conditioning or cold water. At presentation, she also reported shortness of breath, a burning sensation on her feet and scalp, subjective proximal muscle weakness, dysphagia, and weight gain. Her physical examination was notable for periungual telangiectasias, scale, and erythema on the V of her neck area, her extensor arms, and around her eyes (Fig 1). She believed that the erythema on the V of her neck area started 30 years ago, indicating the possibility that she had had potentially undiagnosed amyopathic dermatomyositis (DM) for years before her diagnosis of erythromelalgia. The patient's basic laboratory workup and immunologic studies were unrevealing, and a myositis panel was negative. Muscle magnetic resonance imaging and electromyography were not performed because of the absence of muscle pain or objective weakness on physical examination. Two skin biopsy specimens from the upper portion of the arm and the dorsal surface of the hand showed interface dermatitis (Fig 2), consistent with a diagnosis of DM based on clinicopathologic correlation with typical features of DM (as opposed to lupus, drug reaction, viral exanthem, lichen planus, or graft versus host disease). Pulmonary function testing showed an obstructive pattern with decreased carbon monoxide diffusion in the lung (62%), and chest computed tomography (CT) showed stable interstitial lung changes. The malignancy workup (including age-appropriate screening and CT of the chest, abdomen, and pelvis) was negative. The patient has had some relief from the burning sensation on her feet from topical 5% lidocaine and petroleum jelly. Because of shared decision-making and risk-benefit analysis, the patient has not started immunosuppressive therapy for her DM, but she is being followed closely. Open in a separate window Fig 1 Patient 1. Swelling and erythema on bilateral hands and feet.

Dermatomyositis (DM) is a rare inflammatory disorder commonly manifesting with proximal muscle weakness and characteristic skin findings. Classic findings include erythematous papules or macules over the small joints of the hand, periungual changes, pruritic/erythematous skin on areas including the scalp, chest, upper back, and other surfaces, as well as interstitial lung disease. Anasarca1 and chronic hyponatremia2 have rarely been reported in cases of DM, and have only been reported separately. Here, we present a case of dermatomyositis associated with both anasarca and hyponatremia.

Tracheobronchial lesions are rare extramuscular complications for idiopathic inflammatory myopathies including dermatomyositis. We herein report a 65-year-old woman with tracheal ulcer during the progression of dermatomyositis-associated interstitial lung disease. Treatment with corticosteroids combined with immunosuppressive agents resulted in improvement of the tracheal ulcer and pulmonary involvement. We believe that the tracheal ulceration might reflect the disease behaviour of dermatomyositis and dermatomyositis-associated interstitial pneumonia.

The connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren’s syndrome (SS), and mixed connective tissue disease (MCTD). In RA patients in particular, interstitial lung abnormality (ILA) (of varying degrees; severe vs. mild) is reported to occur in approximately 20–60 % of individuals and CT disease progression occurs in approximately 35–45 % of them. The ILAs have been associated with a spectrum of functional and physiologic decrement. The identification of progressive ILA may enable appropriate surveillance and the commencement of treatment with the goal of improving morbidity and mortality rates of established RA-ILD. Subpleural distribution and higher baseline ILA/ILD extent were risk factors associated with disease progression. At histopathologic analysis, connective tissue disease-related interstitial lung diseases (CTD-ILDs) are diverse and include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), apical fibrosis, diffuse alveolar damage (DAD), and lymphoid interstitial pneumonia (LIP). Even though proportions of ILDs vary, NSIP pattern accounts for a large proportion, especially in PSS, DM/PM and MCTD, followed by UIP pattern. Evidence has been published that treatment of subclinical CT lung abnormalities showing a tendency to progress to ILD may stabilize the CT alterations. The identification of subclinical lung abnormalities can be appropriate in the management of the disease and CT appears to be the gold standard for the evaluation of lung parenchyma.

A 73-year-old woman who visited our hospital complaining of dry cough for three months was refractory to antimicrobial therapy. Chest computed tomography revealed subpleural consolidation. Specimens obtained from surgical lung biopsy revealed subpleural perilobular airspace organization and fibrosis. After the biopsy, mechanic's hand and Gottron's papules appeared, and anti-melanoma differentiation-associated gene 5 (MDA5) antibody was found to be positive. Subsequently, anti-MDA5 antibody measured in cryopreserved serum from her first admission proved to be positive. It is difficult to suspect the presence of anti-MDA-5 antibody in patients with interstitial lung disease without typical dermatomyositis symptoms or slow disease progression.

Patients with dermatomyositis (DM) and autoantibodies against melanoma differentiation–associated protein 5 (MDA5) are likely to subsequently have clinically amyopathic dermatomyositis and interstitial lung disease (ILD).1, 2, 3 These patients characteristically present classical cutaneous manifestations of DM in addition to specific findings such as palmar papules and skin ulceration, especially at the digital pulp.1, 2, 3 We present a patient with anti-MDA5–positive DM with toxic epidermal necrolysis (TEN)-like eruption.

Polymyositis (PM) and dermatomyositis (DM) are different disease subtypes of idiopathic inflammatory myopathies (IIMs). The main clinical features of PM and DM include progressive symmetric, predominantly proximal muscle weakness. Laboratory findings include elevated creatine kinase (CK), autoantibodies in serum, and inflammatory infiltrates in muscle biopsy. Dermatomyositis can also involve a characteristic skin rash. Both polymyositis and dermatomyositis can present with extramuscular involvement. The causative factor is agnogenic activation of immune system, leading to immunologic attacks on muscle fibers and endomysial capillaries. The treatment of choice is immunosuppression. PM and DM can be distinguished from other IIMs and myopathies by thorough history, physical examinations and laboratory evaluation and adherence to specific and up-to-date diagnosis criteria and classification standards. Treatment is based on correct diagnosis of these conditions., High lights • Challenges of diagnosis and management influences the clinical research and practice of Polymyositis and dermatomyositis. • Diagnostic criteria have been updated and novel therapies have been developed in PM/DM. • Pathogenesis investigation and diagnosis precision improvement may help to guide future treatment strategies.

Atypical hydroa vacciniforme (HV) is a rare vesiculopapular eruption reported mainly in patients from Asia and Latin America1, 2, 3 and presents with similar but more severe cutaneous manifestations than classic HV. In addition, systemic findings may be present.1 Distinguishing between classic and atypical HV is essential, as the latter carries a worse prognosis and may progress to HV-like lymphoma.1, 2 Involvement of muscle resulting in a picture similar to dermatomyositis (DM) is a rarely reported presentation of atypical HV.4, 5 Herein, we present the first case, to our knowledge, of a 9-year-old Lebanese girl with atypical HV presenting with a picture of DM.

Summary Background Acute exacerbation (AE) is currently established as a distinct condition with acute deterioration of respiratory status in idiopathic pulmonary fibrosis (IPF). Recently, several studies have reported that AE also occurred in interstitial pneumonias other than IPF, such as collagen vascular disease-associated interstitial pneumonia (CVD-IP). However, the incidence of AE in CVD-IP and its clinical characteristics remain to be fully determined. This study was conducted to elucidate cumulative incidence of AE in CVD-IP and its clinical features. Methods We reviewed 83 biopsy-proven CVD-IP patients, estimated cumulative incidence of AE, and examined its clinical characteristics. Results Among 83 CVD-IP patients, six patients with a mean age of 65.7 years developed AE (overall incidence, 7.2%; 1-year incidence, 1.25%). Underlying CVDs included rheumatoid arthritis (RA) ( n =5; overall incidence, 20.0%) and primary Sjogren syndrome ( n =1; overall incidence, 5.9%). Patients with AE showed acute respiratory deterioration with severe hypoxemia (mean PaO 2 /FiO 2 ratio, 131). Radiologically, ground-glass opacity was superimposed on the underlying reticular abnormalities. Preexisting histological patterns included three usual interstitial pneumonia (UIP) and two non-specific interstitial pneumonia (NSIP). Five (83.3%) of six patients died of respiratory failure despite intensive therapy. Univariate Cox's proportional hazards analysis showed that age and RA diagnosis were significantly associated with AE. Multivariate Cox's proportional hazards analysis indicated that age was an independent significant factor predicting AE. Conclusions These data suggest that AE can occur in CVD-IP, and this condition is closely similar to that of IPF with poor prognosis. AE is most common in RA, and associated with higher ages.