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Master thesis

Dermatomyosite avec nécrose cutanée digitale extensive et pneumopathie interstitielle diffuse associée aux anticorps anti-MDA-5

Abstract : Introduction. Dermatomyositis (DM) is an inflammatory disease associated with autoantibodies in 50 to 70% of cases. A new antibody anti MDA-5 has been described in association with a specific type of DM with a rapidly interstitial lung disease andminimal muscle disease. We report the first case of DM with MDA-5 antibodies withinterstitial lung disease and a massive digital necrosis.Observation. A 28 years old male was hospitalized for asthenia, myalgia and subacutedyspnea. Examination demonstrated skin lesion with edema of every digit associatedwith purpurique and cyanotic lesion. Were also noticed erythematous papules of helix,elbow and Gottron’s papules. A steroid therapy was initiated. The immunoprecipitationresults indicated the presence of anti MDA-5 antibodies. Despite the corticosteroid, therespiratory status gradually deteriorated towards a pulmonary fibrosis and a rapidlyextensive necrosis appears on every fingers and toes. Theses affects were resistant tocyclophosphamide, immunoglobulin and get stabilized by ciclosporin.Discussion. Antibody anti MDA-5 are specific of DM and are a risk factor of interstitiallung disease (70% of cases) with a higher risk of mortality (40%). The cutaneouspresentation of this DM is specific with palmar papules and mucocutaneous ulceration.Rapidly digital necrosis have not been previously reported. None of treatments hasshown superiority.Conclusion. We report the first case of DM with MDA-5 antibodies with interstitial lungdisease and a massive digital necrosis. Because of the pulmonary risk and in front ofclinical lesions of MDA-5 DM, those antibodies should be looked for
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https://hal.univ-lorraine.fr/hal-01732847
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Laurent Charbit. Dermatomyosite avec nécrose cutanée digitale extensive et pneumopathie interstitielle diffuse associée aux anticorps anti-MDA-5. Sciences du Vivant [q-bio]. 2015. ⟨hal-01732847⟩

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