A. R OSEN * S. T HIMON * D. T ERNANT   M.C. M ACHET à § G. P AINTAUD  § L. M ACHET *§ *Service de Dermatologie,  Laboratoire de Pharmacologie, àService d’Anatomie Pathologique, Centre Hospitalier Universitaire, Tours, France §Universite ´ Franc ¸ois-Rabelais de Tours, Tours, France Correspondence: L. Machet E-mail: machet@med.univ-tours.fr References 1 Shih T, Lindley C. Bevacizumab: an angiogenesis inhibitor for the treatment of solid malignancies. Clin Ther 2006; 28:1779–802. 2 Azizi AA, Haberler C, Czech T et al. Vascular-endothelial-growth-fac- tor (VEGF) expression and possible response to angiogenesis inhibi- tor bevacizumab in metastatic alveolar soft part sarcoma. Lancet Oncol 2006; 7:521–3. 3 Herbst RS, Johnson DH, Mininberg E et al. 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A first case report of a patient with paraneoplastic dermatomyositis developing diffuse alveolar haemorrhage DOI: 10.1111/j.1365-2133.2010.09800.x MADAM, Dermatomyositis (DM) and polymyositis (PM) are in- flammatory myopathies, associated with interstitial lung dis- ease (ILD) in 5–30% of cases. 1 The occurrence of diffuse alveolar haemorrhage (DAH) in the course of DM is excep- tional. We present a case of DAH in the context of a paraneo- plastic DM, successfully treated with steroids and immunosuppressive treatment. A 67-year-old woman presented with myalgia and severe weakness of the limbs associated with an oedematous ery- thema of the face, neck, elbows and dorsum of fingers evolving for 1 month. The muscle strength testing score of the proximal muscles was £ 3. Serum levels of muscle- associated enzymes were considerably elevated: creatine phos- phokinase 9Æ142 IU L )1 (normal < 140 IU L )1 ); aldolase 100 IU L )1 (normal < 7Æ6 IU L )1 ); lactate dehydrogenase 2145 IU L )1 (normal < 439 IU L )1 ). Immunological investi- gations showed positive antinuclear antibodies at 1 ⁄ 2500 on mouse liver extracts with spotted fluorescence, positive anti- Mi2 antibodies, and negative anti-DNA, anti-JO-1, anti-PL-7, anti-PL-12, anti-ENA, perinuclear and cytoplasmic antineutro- phil cytoplasmic antibodies (ANCA) and antiglomerular base- ment membrane antibodies while complement levels were normal. Two days after admission, she developed recurrent haemoptysis and resting dyspnoea with bilateral crackles on lung auscultation. Chest X-rays and thoracic computed tomography (CT) scan showed diffuse ground-glass opacities with basal predominance (Fig. 1a). Bronchoscopy revealed bilateral active bleeding in the airways, while bronchoalveo- lar lavage (BAL) was bloody and cell count showed 40% of siderophages. Cultures of BAL fluid were negative for bacte- ria, viruses, fungi and mycobacteria. She was treated with high-dose steroids (three daily intravenous pulses of 500 mg methylprednisolone, followed with prednisone 1Æ5 mg kg )1 daily). Intravenous cyclophosphamide pulses were also given (750 mg m )2 every 3 weeks for 6 months). Respiratory func- tion improvement was obtained in 3 weeks, muscle strength was restored and the skin rash had disappeared after 3 months of treatment. Search for an underlying malignancy was done after stabilization of the respiratory function, identifying a colon adenocarcinoma, which was surgically resected 4 months after the onset of disease. Steroids were progressively tapered and reduced to 15 mg daily after a 10-month follow- up without cutaneous, muscular or pulmonary recurrence. However, crackles were still heard, diffusing capacity of the lung for carbon monoxide was 61% of the predicted value and a thoracic CT scan displayed bibasal fibrous reticulations sug- gesting residual underlying ILD (Fig. 1b). The diagnosis of DM was highly probable in our patient according to the criteria established by Bohan and Peter. 2 Because the DM occurred in association with colonic malig- nancy, it was classified as a paraneoplastic DM. Independently of the positivity of antinuclear antibodies and of anti-Mi2 anti- bodies, highly specific for DM in the context of myositis, 3 no other clinical or biological evidence for an associated connec- tive tissue disease could be found. Pulmonary manifestations in inflammatory myositis are usu- ally secondary to respiratory muscle failure or ILD. If ILD is a well-established feature of lung involvement in DM, 1 DAH is considered as exceptional. It may be observed in noninflam- matory diseases, such as cardiac failure, dyscrasia, drug intake or lung involvement with cancer. Nevertheless, systemic inflammatory diseases are the leading causes of DAH, includ- ing mostly ANCA-associated small-vessel vasculitis, connective tissue disorders (systemic lupus erythematosus, mixed connec- tive tissue disease, scleroderma) and antiglomerular basement membrane antibody disease. 4,5 In the setting of inflammatory myositis, DAH has been described very rarely. Schwarz et al. 6 reported two cases occurring in patients with PM. Two cases in patients diagnosed with mixed connective tissue disease associating inflammatory myositis have been described 7,8 and one case of recurrent DAH occurring in juvenile DM has been published. 9 To our knowledge, DAH has not been reported previously in the course of paraneoplastic DM in adults. DAH results from injury of the alveolar-capillary membrane, leading to acute respiratory insufficiency due to alveolar flooding 4 and Ó 2010 The Authors Journal Compilation Ó 2010 British Association of Dermatologists • British Journal of Dermatology 2010 163, pp208–234 Correspondence 227