["Mechanic's hands" during antisynthetase syndrome: a marker of systemic involvement?].

BACKGROUND: Antisynthetase syndrome is characterized by the presence of interstitial lung disease, inflammatory myopathy, joint disease, Raynaud's phenomenon and characteristic skin lesions of the hands known as "mechanic's hands" associated with the presence of serum antibody to aminoacyl transfer-RNA synthetases. We report the case of a patient in whom cutaneous relapse consistently preceded CT evidence of pulmonary fibrosis flare-up. PATIENT AND METHODS: A 56-year-old patient consulted for recent exertional dyspnoea and dry cough. Clinical examination showed the skin of the tips and edges of the fingers to be thickened, hyperkeratotic and fissured. High-resolution computed tomography thoracic scans revealed interstitial lung lesions affecting both lower lobes. Lab tests showed elevated serum creatine kinase and positivity for anti-Jo-1 antibodies. Response to treatment with corticosteroids and cyclophosphamide was marked by an improvement in symptoms and regression of radiological anomalies together with disappearance of cutaneous signs. Nine months later, after changeover from cyclophosphamide to mycophenolate mofetil, cutaneous relapse and flare of interstitial lung disease were observed. A second course of cyclophosphamide followed by azathioprine resulted in regression of the cutaneous and pulmonary lesions.
CONCLUSION: Mechanic's hands may be considered a marker of visceral involvement and should be sought in any instance of suspected antisynthetase syndrome flare-up.