Amyopathic Dermatomyositis Complicated by Pneumomediastinum

Dermatomyositis is an inflammatory disease of unclear etiology with characteristic cutaneous and musculoskeletal findings. Amyopathic dermatomyositis is a subtype without musculoskeletal involvement. Many cases of dermatomyositis are associated with underlying malignancy, but pulmonary manifestations can also be seen, the most common of which is interstitial lung disease. Pneumomediastinum is a rare complication that is important for clinicians to recognize, as it may be fatal if left untreated. The sudden onset of facial edema and shortness of breath in the setting of dermatomyositis should raise the suspicion of this condition.A 52-year-old Caucasian woman presented to the authors’ clinic with a six-month history of fatigue, hair loss, and joint stiffness of the ankles, wrists, and knees. On physical exam, the patient was noted to have periorbital erythema with profound edema (Figure 1). Confluent and violaceous erythema was noted over the upper chest, and violaceous, ulcerated plaques were present on the metacarpalphalangeal joints, distal interphalangeal joints, and elbows bilaterally (Figure 2). A punch biopsy of the left elbow plaque was performed at the time. Open in a separate windowFigure 1Significant facial and neck swelling of acute onsetOpen in a separate windowFigure 2Erythematous, ulcerated papules overlying the MCP, PIP, and DIP joints of the handsThe patient was started on prednisone 60mg once daily for a presumptive diagnosis of dermatomyositis (DM), and a comprehensive laboratory evaluation was ordered. The biopsy results were consistent with DM, and anti-Jo-1 antibody levels were normal. As her creatine kinase and aldolase levels were also normal, a diagnosis of amyopathic dermatomyositis (ADM) was made. At the next office visit, the patient showed no signs of clinical improvement, so hydroxychloroquine 200mg twice daily and clobetasol ointment were added. This combination led to significant improvement in the patient’s cutaneous lesions.One year after initial presentation, the patient came to the authors’ clinic with an acute episode of shortness of breath as well as facial and neck edema. Crepitus was appreciated on physical exam. She was sent to the nearest emergency room where a computed tomography scan revealed bilateral pneumomediastinum (PnM) with subcutaneous emphysema. Bilateral chest tubes were placed at the time and cyclosporine therapy was initiated. The tubes were removed and the patient was discharged one week later after significant improvement in her condition.     

Juvenile dermatomyositis

Basic aim of this paper is presentation of probable epidemiological characteristics of juvenile dermatomyositis (JDM) in children of Croatia, due to the fact that severity of condition in majority of patients require treatment in our Department of Pediatrics, KBC Zagreb. Our intention is to present guidelines of current diagnostic approach from recent literature, with special accent on therapy. JDM was previously considered as infaust disease, while current therapy has favourable prognosis for complete resolution, as the only one mesenchymopathy with such prognosis. In period between 1988 and 1999 we treated 18 patients with JDM in our Department of Pediatrics. Thirteen cases were male and 5 female (2.6:1). Thirteen patients had clinical course of isolated JDM (72%), while five had JDM inside overlap syndrome (with other mesenchymopathies) (28%). Average age at time of diagnosis for whole group was 10 years (6-14), for girls 10.6 years (6-14) and boys 8.4 years (7-10). At the present time 5 patients (28%) are considered as cured, 7 patients are in remission with low dosis of steroids (39%) and one girl had relaps of JDM after therapy stopage. Three children die (16%), one girl due to respiratory failure and two girls with pulmonary embolia. Two girls have some signs of JDM with dominant clinical signs of sistemic sclerodermia and one girl has signs of generalized morphea. One boy has dominant muscular calcinosis with contractures of large joints, despite treatment and normal laboratory findings. JDM was not discussed in rheumatic literature in Croatia for more then 15 years.     

Juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is an important subtype of dermatomyositis characterized by inflammation of muscle, skin and gastrointestinal tract. A 14-year-old girl, with a history of fever, joint pain, easy fatigability and a rash since the age of 3 years is described. Physical examination, laboratory evaluation, electromyography (EMG) and muscle biopsy were suggestive of a chronic inflammatory process involving the muscles, most likely dermatomyositis. The report highlights the importance of a muscle biopsy as the gold standard for diagnosing dermatomyositis.     

Ureteral necrosis in dermatomyositis

We describe a child with dermatomyositis and calcified necrosis in the middle third of both ureters. Histopathological examination showed vasculitis associated with ureteral necrosis and calcification. These findings together with a similar previous report in the literature have prompted us to correlate dermatomyositis in childhood with ureteral necrosis and to anticipate specifically a lesion in the middle third of the ureter because of the relative lack of blood flow in that segment.     

Actuality of juvenile dermatomyositis

Juvenile dermatomyositis is a rare disorder, but remains the most commonly occurring chronic inflammatory myopathy among children. Other than the proximal muscles and skin, which are routinely affected, vasculopathy may affect other viscera and can be multisystemic. A redefinition of the diagnostic criteria is currently underway and is likely to lead to other clinical signs and to sensitive and non-invasive examinations such as MRI. The impact of juvenile dermatomyositis on health and quality of life remains significant despite systemic corticosteroid therapy and immunosuppressor treatment, which have considerably improved the prognosis. Numerous predictors for favourable and pejorative evolution have been identified. The standardisation and the generalisation of clinical assessment tools will make it possible to carry out the clinical trials required to determine the relevance of the new therapeutic options available for children.     

Ureteral cancer associated with dermatomyositis

Dermatomyositis is a rare inflammatory myopathy that has characteristic cutaneous lesions. Although many malignancies are associated with dermatomyositis, urogenital malignancies have rarely been reported to be associated with dermatomyositis. We report here on the first case of ureteral cancer associated with dermatomyositis. A 42-year-old man presented to us with a skin rash. A clinical diagnosis of dermatomyositis was made due to the skin lesions, muscle weakness, arthralgia, the increased erythrocyte sedimentation rate and the increased creatine kinase level. The patient revealed microscopic hematuria and abnormal urine cytology during the investigation for the underlying malignancy. Retrograde pyelography demonstrated a suspicious lesion in the right mid-ureter, and the ureteroscopic biopsy revealed the urothelial carcinoma. Although an operation was recommended, the patient died of pneumonia associated with his interstitial lung disease, which is one of the poor prognostic indicators of dermatomyositis.     

Neuromuscular blockade using vecuronium in dermatomyositis

The significant features of neuromuscular blockade with vecuronium in a patient with dermatomyositis are described: vecuronium 0.08 mg/kg resulted in 90%, 0.12 mg/kg in 100% neuromuscular blockade. In contrast to claims made in some previous publications, dermatomyositis did not produce increased sensitivity to vecuronium. Onset time and duration of action were also within normal limits in our patient. Time of spontaneous recovery until antagonism with neostigmine was markedly prolonged, but the dermatomyositis was only one of various possible explanations. Although there are potential hazards in the use of neostigmine in patients with dermatomyositis, antagonism of the neuromuscular block with 2 mg neostigmine was without problems in our patient. Our data support recent suggestions to reconsider the implications of dermatomyositis for anesthesia.     

Anaesthetic management of a child with dermatomyositis

A two years, ten months old male with dermatomyositis was anaesthetized with enflurane, nitrous oxide and oxygen by mask followed by intravenous succinylcholine to facilitate endotracheal intubation. The evoked thumb twitch in response to succinylcholine demonstrated an abnormal, short-lived contracture. The depression, duration and return to control of muscle twitch tension and a transient rise in serum potassium concentration followed a normal pattern.     

Clinical and autoantibody phenotypes of juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a heterogeneous autoimmune inflammatory myositis with symmetrical proximal muscle weakness and a characteristic rash. Juvenile dermatomyositis is characterized by variable presentation and phenotypes. Detection of myositis autoantibodies is useful in improving JDM diagnosis and predicting the prognosis.In this literature review based on case series we analyze clinical and autoantibody phenotypes of JDM in four patients who were hospitalized in one regional center in Ukraine during the last 3 years and three of them presented in the time of the COVID-19 pandemic. The reviewed literature showed the last updates for the JDM diagnosis and the role of myositis autoantibodies in the prediction of disease course, systemic involvement, and malignancy risk.The presence of anti-synthetase syndrome in all presented patients, mainly due to anti-PL-7 autoantibodies, encourages further study with more patients and with detection of other myositis-specific autoantibodies to identify or refute certain regional features.     

Paraneoplastic dermatomyositis as presentation of thymic carcinoma

Thymic carcinomas are very rare and heterogeneous groups of anterior mediastinum neoformations with an extremely aggressive behavior. Often, the diagnosis is made in the advanced stages. Paraneoplastic syndromes associated with thymic carcinoma are extremely rare. We report a case of a 64-year-old man presenting with early stage thymic carcinoma which was discovered because of associated paraneoplastic dermatomyositis. The dermatomyositis disappeared completely after radical resection of the tumor. After 20-month follow-up, the patient is in good clinical condition without recidivism of disease.