2012
DOI: 10.1159/000346573
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Response of Mucocutaneous Lesions to Rituximab in a Case of Melanoma Differentiation Antigen 5-Related Dermatomyositis

Abstract: We report the first case in Western Europe of a person presenting with dermatomyositis associated with melanoma differentiation antigen 5 antibodies. She sequentially developed severe mucocutaneous erythematous and itchy lesions of the face, scalp, neck, knees and recurrent aphthae. In addition she presented painful, periungual, edematous digital lesions and small ulcers with digital necrosis. Her rapidly evolving, near-fatal interstitial lung disease responded to high-dose intravenous cyclophosphamide. Howeve… Show more

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Cited by 26 publications
(26 citation statements)
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References 31 publications
(54 reference statements)
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“…Moreover, a subsequent subgroup analysis has highlighted that response to rituximab may be influenced by serological phenotype with ASS and antiMi2 patients more likely to gain benefit [122]. A therapeutic role for rituximab within the different clinicoserological phenotypes has been further emphasised in a number of series, including refractory patients with ASS-ILD, anti-MDA5 and anti-SRP disease [117,[123][124][125][126][127][128][129]. Therefore, it seems that stratification based on autoAbs can act as predictive markers of clinical response to treatment, which again highlights their diagnostic utility.…”
Section: Does Clinico-serological Phenotype Influence Outcome and Trementioning
confidence: 99%
“…Moreover, a subsequent subgroup analysis has highlighted that response to rituximab may be influenced by serological phenotype with ASS and antiMi2 patients more likely to gain benefit [122]. A therapeutic role for rituximab within the different clinicoserological phenotypes has been further emphasised in a number of series, including refractory patients with ASS-ILD, anti-MDA5 and anti-SRP disease [117,[123][124][125][126][127][128][129]. Therefore, it seems that stratification based on autoAbs can act as predictive markers of clinical response to treatment, which again highlights their diagnostic utility.…”
Section: Does Clinico-serological Phenotype Influence Outcome and Trementioning
confidence: 99%
“…It has been used with success in some autoimmune diseases, non-Hodgkin lymphomas and, recently, in pemphigus vulgaris [9,10]. Initial successful administration in pemphigus was reported in a patient with follicular non-Hodgkin lymphoma and paraneoplastic pemphigus [11].…”
Section: Introductionmentioning
confidence: 99%
“…Our findings were in line with those results in that the anti-PL-12 antibody-positive patient survived. However, there are few reports describing the successful treatment of anti-MDA5 antibody-positive patients with RTX (12-14). In our report, in spite of intensive care, including the administration of RTX, the 2 anti-MDA5 antibody-positive patients died.…”
Section: Discussionmentioning
confidence: 99%
“…During the past year, some case series showed the beneficial effects of rituximab (RTX) treatment in anti-ARS antibody-positive DM patients (7-11). In addition, an anti-MDA5 antibody-positive patient displaying the mucocutaneous manifestations of DM who was refractory to IVCY treatment but who improved after the administration of RTX (12), and the successful treatment of 2 anti-MDA5 antibody-positive patients with RTX were recently reported (13,14). However, the efficacy of RTX treatment in DM patients with RP-ILD, including anti-MDA5 antibody-positive patients is still not clear.…”
Section: Introductionmentioning
confidence: 99%