title: [Extensive digital necrosis during dermatomyositis associated with MDA-5 antibodies].
abstract: BACKGROUND
  Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis.
  
  
  METHODS
  A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine.
  
  
  CONCLUSIONS
  Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority.
  
  
  CONCLUSIONS
  We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out.
authors.affiliation: Département de dermatologie et allergologie, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France. Electronic address: l.charbit@chu-nancy.fr.
authors.forename: L
authors.initials: L
authors.lastname: Charbit
authors.affiliation: Département de dermatologie et allergologie, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: A-C
authors.initials: AC
authors.lastname: Bursztejn
authors.affiliation: Département de médecine interne, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: S
authors.initials: S
authors.lastname: Mohamed
authors.affiliation: Département de médecine interne, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: P
authors.initials: P
authors.lastname: Kaminsky
authors.affiliation: Département de dermatologie et allergologie, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: B
authors.initials: B
authors.lastname: Lerondeau
authors.affiliation: Département de dermatologie et allergologie, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: A
authors.initials: A
authors.lastname: Barbaud
authors.affiliation: Département de médecine interne, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: J
authors.initials: J
authors.lastname: Deibener-Kaminsky
authors.affiliation: Département de dermatologie et allergologie, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Schmutz
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D003882:Dermatomyositis / Q000150:complications / Q000276:immunology*; D005385:Fingers / Q000473:pathology*; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1 / Q000276:immunology*; D017563:Lung Diseases, Interstitial / Q000276:immunology; D008297:Male; D009336:Necrosis; D012867:Skin / Q000473:pathology*
chemical_list: D001323:Autoantibodies; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1
keywords: Anti-MDA-5 antibody; Anticorps anti-MDA-5; Dermatomyosite; Dermatomyositis; Digital necrosis; Interstitial lung disease; Nécrose digitale; Pneumopathie interstitielle diffuse