Becker muscular dystrophy (BMD) is a neuromuscular disorder allelic to Duchenne muscular dystrophy (DMD), caused by in-frame mutations in the dystrophin gene, and characterized by a clinical progression that is both milder and more heterogeneous than DMD. Muscle magnetic resonance imaging (MRI) has been proposed as biomarker of disease progression in dystrophinopathies. Correlation with clinically meaningful outcome measures such as North Star Ambulatory Assessment (NSAA) and 6 minute walk test (6MWT) is paramount for biomarker qualification. In this study, 51 molecularly confirmed BMD patients (aged 7–69 years) underwent muscle MRI and were evaluated with functional measures (NSAA and 6MWT) at the time of the MRI, and subsequently after one year. We confirmed a pattern of fatty substitution involving mainly the hip extensors and most thigh muscles. Severity of muscle fatty substitution was significantly correlated with specific DMD mutations: in particular, patients with an isolated deletion of exon 48, or deletions bordering exon 51, showed milder involvement. Fat infiltration scores correlated with baseline functional measures, and predicted changes after 1 year. We conclude that in BMD, skeletal muscle MRI not only strongly correlates with motor function, but also helps in predicting functional deterioration within a 12-month time frame.

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Pemphigus vulgaris is an autoimmune bullous disease affecting the skin and mucosa. It is caused by autoantibodies targeting the adhesion molecules desmoglein 1 and desmoglein 3, which, respectively, mediate adhesion between keratinocytes in skin and mucosa, resulting in the formation of blisters and erosions. Early recognition and treatment are crucial for remission of this disorder. Treatment for severe pemphigus traditionally includes long-term corticosteroids and immunosuppressants [1]. Rituximab, a chimeric monoclonal antibody against the CD20 antigen of B lymphocytes, was recently introduced and has proven to be effective in resistant and life-threatening pemphigus [2]. Protocols for administration of rituximab are determined for use in non-Hodgkin lymphoma (375 mg/m 2 once a week, with duration depending on the type of lymphoma) and for rheumatoid arthritis (2 doses of 1000 mg given 2 weeks apart) [3]. A lower dose of rituximab (500 mg at 2 weeks interval) can be used in pemphigus [4][5][6].A 67-year-old woman was referred to our dermatology department for recent onset of erosive lesions of the oral cavity. The patient complained of severe difficulty swallowing, forcing her to feed almost exclusively with a liquid diet. The skin was not involved, showing only signs of previously active lesions. She denied trauma and application of topical drugs on the areas involved. Her medical history did not reveal any association with other immune-mediated diseases. Evaluation of circulating anti-DSG1, anti-DSG3, and anti-BP180 antibodies revealed a high anti-DSG3 autoantibody titer (192.13 U/mL, normal value <7 U/mL), which is consistent with a diagnosis of pemphigus vulgaris. Treatment was initially started with intravenous methylprednisolone 80 mg/d for 2 weeks, which was thereafter slowly tapered to 40 mg/d over 2 weeks, with complete remission. The patient was then treated with oral prednisone 50 mg/d for 1 month and 25 mg/d for a further month. At this time, the disease relapsed, with oral involvement only, and azathioprine 100 mg/d was combined with prednisone 25 mg/d for 3 months. The oral lesions did not resolve. Consequently, we decided to administer rituximab [7] in a 2-dose regimen (2 weeks apart) of 500 mg per dose. Prior to infusion of rituximab, the patient was premedicated with intravenous acetaminophen 1000 mg, chlorphenamine 10 mg, and methylprednisolone 20 mg diluted in 100 mL of normal saline. Rituximab 500 mg was then diluted in 500 mL of Manuscript

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