[Extensive digital necrosis during dermatomyositis associated with MDA-5 antibodies]. / Nécroses digitales étendues au cours d'une dermatomyosite avec anticorps anti-MDA-5.

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. PATIENTS AND 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. DISCUSSION: 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. CONCLUSION: 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.

[Clinical relevance of leukocyte differential in patients with marked leukocytosis in the emergency room]. / Signification d'une hyperleucocytose marquée et de la formule sanguine dans les situations d'urgence.

PURPOSE: We analyzed the characteristics of the leukocyte differential and the clinical outcome in patients admitted in an emergency department with marked leukocytosis greater than 20×10(9)G/L. METHODS: We studied a case series of consecutive patients admitted in an emergency department. The medical records were retrospectively reviewed after patient discharge. Three groups were defined: patients with infectious disorders (group I), noninfectious disorders (group II), and trauma (group III). Admission in intensive care unit (ICU), consciousness impairment or death defined the subgroup S of high severity. RESULTS: Groups I, II and III comprised, respectively, 150, 95 and 86 patients. The group I presented with higher temperature and neutrophilia (22,2±4.9 vs 20.9±4.0 and 21.1±3.9×10(9)G/L; P<0.001), and more profound eosinopenia (0.058±0.094 versus 0.098±0.170 and 0.092±0.104×10(9)G/L; P<0.001) and lymphopenia (1.16±0.98 vs 1.53±1.04 and 1.73±1.10×10(9)G/L; P<0.001) than the two other groups. Both neutrophilia and lymphopenia were independent predictors of infection by multivariate analysis. Frequencies of admission in ICU were, respectively, 8.7%, 40% and 43% (P<0.001). Leukocyte and neutrophil counts were significantly higher and basophil count significantly lower in subgroup S. Overall, 13.6% of the patients died and were characterized by basopenia. CONCLUSION: Marked leukocytosis indicated severe illness. Lymphopenia, eosinopenia and temperature were significant predictors of infection. A more severe clinical course was correlated with higher neutrophilia and basopenia.