Development and Evaluation of the Dermatomyositis Outcomes for Muscle and Skin as an Outcome Measure in Dermatomyositis Clinical Trials.

The Total Improvement Score (TIS), which is used as the primary efficacy measure in dermatomyositis (DM) clinical trials, lacks a skin-specific measure. However, skin is a defining feature of DM. In this study, data were analyzed from the phase 3 trial of lenabasum in DM.

Clinical characteristics and symptom progression of dermatomyositis subtypes: A retrospective analysis of a prospective database.

Background: Disease characteristics of classic dermatomyositis (DM) and clinically amyopathic DM (CADM) are well established, but there exists limited knowledge on the disease progression of these subtypes.

Objective: The objective of this study was to longitudinally track and characterize classic DM and CADM patients who experience changes in disease presentation.

Methods: We conducted a retrospective review of prospectively collected data on 269 DM patients from a longitudinal database.

Emerging immunotherapeutic strategies for cutaneous lupus erythematosus: an overview of recent phase 2 and 3 clinical trials.

Introduction: Cutaneous lupus erythematosus (CLE) is an autoimmune disease that is clinically heterogenous and may occur with or without the presence of systemic lupus erythematosus (SLE). While existing on a spectrum, CLE and SLE present differences in their underlying pathogenesis and therapeutic responses. No new therapies have been approved in recent decades by the U.

The physical and emotional impact of cutaneous dermatomyositis: a qualitative study.

Dermatomyositis (DM) is a rare autoimmune disease characterized by distinctive cutaneous manifestations, often accompanied by muscle inflammation and interstitial lung disease. DM has a significant impact on quality of life (QoL) in patients, due to the physical and emotional symptoms caused by their disease. Despite this known emotional impact, there is no published literature capturing how adults with DM feel about their disease, from their perspective.

Current concepts and advances in dermatomyositis: a dermatological perspective.

Dermatomyositis (DM) is an autoimmune disorder in which clinically amyopathic DM, characterised by hallmark cutaneous findings in the absence of clinical weakness, represents 20% of patients. This review will highlight current concepts and recent advances made in DM from a dermatological perspective, with a discussion of skin-predominant DM and its distinct challenges regarding diagnosis and management as well as their implications in clinical trials. An update will be presented with respect to classification criteria, pathogenesis in cutaneous DM, myositis-specific autoantibodies and their associations with cutaneous findings, skin-specific outcome measures and new therapeutics with their efficacy in skin disease.

Annular bullous pemphigoid: A case report and review of literature.

Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented.

Bullous Pemphigoid-Associated Mortality Rate in a Canadian Tertiary Referral Centre.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder in adults. Most individuals with BP are over the age of 60. Its worldwide incidence has been increasing owing to population aging.

Verrucous plane xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis: A case report.

Cutaneous xanthomas are the result of dermal deposition of lipid, mostly caused by disorders of lipid metabolism. Less commonly, they occur in the setting of cholestatic liver disease, leading to accumulation of lipoprotein X, a rare form of dyslipidemia that does not respond well to conventional treatments. We describe an atypical presentation of sudden diffuse xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis caused by trimethoprim-sulfamethoxazole hypersensitivity.

Improvement in Hailey-Hailey disease with a combination of low-dose naltrexone and oral magnesium chloride: A case report.

Hailey-Hailey disease is a rare autosomal dominant acantholytic disorder due to mutation in the gene and presents with flaccid blisters in intertriginous regions. Its chronic and relapsing course may negatively impact patients' quality of life. Multiple medical and interventional treatments have been described with various efficacy.

Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary.

Linear cutaneous lupus erythematosus in children-report of two cases and review of the literature: A case report.

Linear cutaneous lupus erythematosus is an unusual presentation of cutaneous lupus following Blaschko's lines. It is described mostly in children and young adults and is usually not associated with systemic involvement. We report two cases of linear cutaneous lupus erythematosus in children who significantly improved after treatment with hydroxychloroquine in combination with topical corticosteroids and tacrolimus.

A case of dermatomyositis with anti-TIF1γ antibodies revealing isolated para-aortic lymphadenopathy metastatic recurrence of endometrial cancer: A case report.

Dermatomyositis is an inflammatory myopathy presenting with characteristic cutaneous eruption and may be accompanied by proximal muscle weakness. Dermatomyositis may represent a paraneoplastic syndrome in 15%-25% of cases and has rarely been associated with endometrial cancer. Herein, we report a case of dermatomyositis with anti-TIF1γ antibodies as the first clinical manifestation revealing isolated para-aortic lymphadenopathy metastatic recurrence of endometrial cancer after 4 years of remission.

Refractory extensive vulvar high-grade squamous intraepithelial lesion in an immunosuppressed transplant patient treated with topical cidofovir: A case report.

High-grade squamous intraepithelial lesion of the vulva is a premalignant condition which may be especially resistant to treatments among immunosuppressed patients. We present our experience with the use of topical cidofovir in a refractory case of extensive vulvar high-grade squamous intraepithelial lesion in a 37-year-old transplant patient. Eighteen cycles of cidofovir over a 2-year period led to a sustained significant improvement, mainly of the mucosal lesions and was well tolerated.

Flagellate Dermatitis in a Child Most Likely Secondary to Doxorubicin.

Flagellate dermatitis, a cutaneous eruption in which the patient appears to have been whipped, has been described with antineoplastic agents and shiitake mushroom ingestion. A 15-year-old girl with metastatic Ewing sarcoma developed pruritic erythematous linear lesions on her trunk that became hyperpigmented over time during her first cycle of chemotherapy with doxorubicin, vincristine, cyclophosphamide, and ganitumab. Flagellate dermatitis was diagnosed based on clinical and histologic findings.