Severe Dermatomyositis Research Articles

S3062 Gastrointestinal Ulceration as a Manifestation of Severe Dermatomyositis

S3062 Gastrointestinal Ulceration as a Manifestation of Severe Dermatomyositis

Gastrointestinal Ulceration as a Manifestation of Severe Dermatomyositis -

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Involvement of Type I Interferon Signaling in Muscle Stem Cell Proliferation During Dermatomyositis.

The idiopathic inflammatory myopathy dermatomyositis is an acquired disease that involves muscle, lung, and skin impairments. Patients with dermatomyositis show a wide range of severity of proximal skeletal muscle weakness, associated with inflammatory infiltrates, vasculitis, capillary dropout, and perifascicular myofiber atrophy. Muscles of patients with dermatomyositis show signs of muscle regeneration. Because muscle stem cells (MuSCs) are responsible for myofiber repair, we wondered whether the proliferative properties of MuSCs are altered in dermatomyositis muscle. We investigated the role of type I interferon (IFN-I) in this process because dermatomyositis is associated with sustained inflammation with high IFN-I levels. MuSCs isolated from normal muscles and those from adult and juvenile patients with dermatomyositis were grown in culture and analyzed in vitro for their proliferating properties, myogenic capacities, and senescence. Gain- and loss-of-function experiments were performed to assess the role of IFN-I signaling in the proliferative capacities of MuSCs. MuSCs derived from 8 adult patients with dermatomyositis (DM-MuSCs) (5 severe form and 3 mild form, established from histologic evaluation), from 3 patients with juvenile dermatomyositis, and from normal muscle were used to analyze their myogenesis in vitro. DM-MuSCs exhibited strongly reduced proliferating capacities as compared with healthy MuSCs (-31% to -43% for mild and severe dermatomyositis, respectively), leading to poor myotube formation (-36% to -71%). DM-MuSCs were enriched in senescent, β-galactosidase-positive cells, partly explaining the proliferation defect. Gain- and loss-of-function experiments were performed to assess the role of IFN-I on the proliferative capacity of MuSCs. High concentrations of IFN-I decreased the proliferation of healthy MuSCs. Similarly, conditioned medium from DM-MuSCs decreased the proliferation of healthy MuSCs (-15% to -22%), suggesting the delivery of an autocrine effector. Pharmacologic blockade of IFN signaling (using ruxolitinib or anti-IFN receptor antibodies) in DM-MuSCs rescued their proliferation up to the control values. These results show that autocrine IFN-I signaling prevents MuSC expansion, leading to muscle repair deficit. This process may explain the persistent muscle weakness observed in patients with severe dermatomyositis.

Severe dermatomyositis presenting as quadriparesis: An unusual sequel in elderly

Dermatomyositis, an idiopathic inflammatory myopathy, is one of the rare causes of limbs paresis. The characteristic feature is skin involvement which differentiates this from other inflammatory myopathy. Here we report a case of severe dermatomyositis as his Creatine Phosphokinase (CPK) level was 32,000 who presented with weakness in all four limbs without any dermatological signs. Keywords: Dermatomyositis, Creatinine kinase, Quadriparesis, Elderly.

134 Severe cutaneous dermatomyositis complicated by progressive multifocal leukoencephalopathy

134 Severe cutaneous dermatomyositis complicated by progressive multifocal leukoencephalopathy

A Case Report on Dysphagia and Edema in an Elderly Patient: Warning Signs for Severe Dermatomyositis

A Case Report on Dysphagia and Edema in an Elderly Patient: Warning Signs for Severe Dermatomyositis

JAK1/2 Inhibitor Ruxolitinib Controls a Case of Chilblain Lupus Erythematosus

JAK1/2 Inhibitor Ruxolitinib Controls a Case of Chilblain Lupus Erythematosus

Severe dermatomyositis with pronounced generalized subcutaneous edema and dysphagia: A rare manifestation of a highly active disease

Severe subcutaneous edema is rare in dermatomyositis (DM). Such patients usually have a highly active disease, which requires aggressive treatment. To date, there are 14 reported cases of generalized edema secondary to adult DM. We described a severe case of DM manifesting generalized edema, oropharyngeal dysphagia, and dysarthria. A 44-year-old female presented with a typical rash of DM, proximal muscle weakness, and marked swelling of the limbs and face. The findings in the skin biopsy, muscle enzymes, and electromyography were consistent with DM. No internal malignancy was detected. After a brief initial response to oral dexamethasone, the patient experienced a sudden worsening of muscle weakness with dysarthria and an inability to swallow even saliva. A magnetic resonance imaging study revealed edema of the subcutaneous tissue and muscles. The symptoms improved gradually in 2 months after intravenous pulse corticosteroid therapy. Generalized subcutaneous edema is a very rare manifestation of DM that can occur as a presenting symptom. It appears to be a hallmark of a severe DM that requires prompt and aggressive treatment. Additional cases are needed to establish guidelines for treatment of this rare variant.

SAT0205 Real Life Experience Suggests Differential Effects of Rituximab on Refractory Dermatomyositis and Polymyositis

BackgroundRituximab has been used for patients with refractory myositis in many centers, including ours. In a large double-blind trial on rituximab in inflammatory myopathies, no significant difference has been found...

Thrombotic Microangiopathy and Purtscher-like Retinopathy as a Rare Presentation of Juvenile Dermatomyositis

Juvenile dermatomyositis is a rare systemic vasculopathy that may sometimes present with acute complications. We report here the case of a 7-year-old boy with severe dermatomyositis associated with thrombocytopenia and blurry vision. The presence of schistocytosis and the secondary occurrence of hemolytic anemia were consistent with a diagnosis of thrombotic thrombocytopenic purpura (TTP). Further investigations demonstrated the association of TTP with muscular microangiopathy and Purtscher-like retinopathy. Retinal and hematologic involvements dramatically improved after the initiation of plasma exchange in emergency. This report emphasizes that early recognition of TTP and prompt plasmapheresis are important in a child with severe juvenile dermatomyositis associated with thrombocytopenia.

Limb edema and anasarca associated with severe dermatomyositis: Report of four cases

Dermatomyositis is an autoimmune disorder that causes proximal muscle weakness and skin changes which include generalized erythema, heliotrope rash and/or Gottron’s papules. Generalized or limb edema is an uncommon manifestation of dermatomyositis. Here, we report four cases who presented with generalized or limb edema, proximal muscle weakness, erythematous skin rash and/or dysphagia. Muscle biopsy revealed perifascicular fiber atrophy, a characteristic finding of dermatomyositis. The absence of other causes indicated that the generalized or limb edema was caused by dermatomyositis. None of our patients showed significant improvement with steroids alone, and more aggressive immunotherapy eventually resolved the edema. We concluded that generalized or limb edema may be a hallmark of a severe form of dermatomyositis and requires prompt and aggressive therapies.

Effective Cyclophosphamide Pulse Therapy for an Young Infant with Severe Dermatomyositis

Effective Cyclophosphamide Pulse Therapy for an Young Infant with Severe Dermatomyositis

111. Limb edema and anasarca associated with severe dermatomyositis: Four case reports

111. Limb edema and anasarca associated with severe dermatomyositis: Four case reports

Severe Dermatomyositis Triggered by Interferon Beta-1a Therapy and Associated With Enhanced Type I Interferon Signaling

Type I interferons (IFNs) are common therapeutics for several diseases, including viral infections and multiple sclerosis (MS). Although numerous studies have implicated type I INFs with the production of autoantibodies and the development of certain autoimmune disorders, interferon beta has not previously been described in association with dermatomyositis, to our knowledge. Previous microarray studies of muscle biopsy specimens from patients with dermatomyositis disclosed a type I IFN-induced gene expression profile. The central role of plasmacytoid dendritic cell precursors, together with increased type I IFN production, suggests a pivotal role for type I IFNs in dermatomyositis. We report a case of dermatomyositis exacerbated or induced by interferon beta therapy for MS and provide evidence that demonstrates enhanced type I IFN signaling in this patient. We observed new-onset dermatomyositis in a 57-year-old patient treated with interferon beta for MS. His symptoms were exacerbated temporally by interferon beta injections. Immunohistochemical staining of skin biopsy specimens for myxovirus-resistance protein A (a surrogate marker for cutaneous type I IFN signaling) showed increased staining that correlated temporally with interferon beta treatment and subsequent disease activity. In vitro treatment with interferon beta of peripheral blood mononuclear cells isolated from our patient revealed enhanced type I IFN signaling assessed by interferon-induced gene expression profiles. To our knowledge, this is the first description of dermatomyositis exacerbated or induced by interferon beta treatment. Our results demonstrate enhanced type I IFN signaling following interferon beta treatment in our patient with dermatomyositis.

Case Report: Dramatic Recovery of Lung Adenocarcinoma–Associated Dermatomyositis with Targeted Lung Cancer Therapy Alone

This case report details the sudden onset of severe dermatomyositis (DM) symptoms followed by rapid progression of adenocarcinoma of the lung and an obvious diminution of the primary tumor with the administration of lung cancer targeted drug therapy alone, followed by nearly complete disappearance of the DM symptoms, with no conspicuous improvement in the DM symptoms when using corticosteroids.

Cerebral phaeohyphomycosis caused by Cladophialophora bantiana and Fonsecaea monophora: report of three cases

Three cases of cerebral phaeohyphomycosis are described. Two cases (Cases 1, 2) are caused by highly neurotropic fungi, Cladophialophora bantiana, and the other one (Case 3) is the first reported case in the United States, caused by the newly defined Fonsecaea monophora. (Case 1): A 65-year-old woman had been treated for a presumed diagnosis of Guillain-Barré syndrome and was found to have a ring-enhancing, fluid-filled lesion in the right frontal lobe. The lesion was aspirated twice and then resected completely. (Case 2): A 45-year-old woman with a history of severe dermatomyositis presented with subacute ischemia in the left brainstem. Approximately 2 months later, she developed acute obstructive hydrocephalus and was found to have small cystic lesions in the left ambient cistern, fourth ventricle and cerebral aqueduct, which had probably caused the previous ischemic symptoms due to emboli/ thrombi. (Case 3): A 62-year-old, post livertransplant woman developed multiple brain and bone abscesses. Cultures from these lesions grew the same fungi. Histologically, all three cases revealed multiple epithelioid and giant cell granulomata with groups of golden-brown yeast-like cells as well as chains of budding cells. In Case 3, scattered muriform cells, characteristic of chromoblastomycosis, were present. In Cases 2 and 3, the fungi were easily identified on frozen sections, which may be considered useful in determining post-operative therapy.

Prostate cancer underlying acute, definitive dermatomyositis: successful treatment with radical perineal prostatectomy

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation leading to progressive weakness of the proximal muscles. In 7-66% of cases of adult dermatomyositis different malignant tumours can promote the difficult cascade mechanisms at the cell level, leading to rapid weakness of skeletal muscles [1]. We report on a patient with all characteristic signs of acute, severe dermatomyositis associated with a low-grade, low-stage prostate cancer cured by radical perineal prostatectomy.

Severe Dermatomyositis Associated with Lymph Node Recurrence of an Endometrial Carcinoma

Severe Dermatomyositis Associated with Lymph Node Recurrence of an Endometrial Carcinoma

Dermatomyositis responding to treatment of associated toxoplasmosis

We report a 59-year-old woman with severe dermatomyositis in association with high serum toxoplasma antibody titres who was successfully treated with pyrimethamine and sulphadiazine. This case supports an aetiological role for toxoplasmosis in the pathogenesis of some cases of dermatomyositis.

Milk of calcium fluid collection in dermatomyositis: Ultrasound findings

Extensive subcutaneous and intermuscular calcium laden fluid collections (milk of calcium) were found in two young girls with severe dermatomyositis. Sonographic examination clearly showed the nature and extent of the collections. Knowledge of this new feature of dermatomyositis should help avoid confusing these fluid collections with soft tissue infection and deep abscess in these often steroid dependent children.