Paraneoplastic sclerodermiform syndrome

• Published in: Revue des maladies respiratoires Vol. 27; no. 3; p. 251
• Main Authors: Hounkpati, A, Marie, I, Paillotin, D, Muir, J-F, Cuvelier, A
• Format: Journal Article
• Language: French
• Published: France 01.03.2010
• Subjects: Adenocarcinoma - complications; Humans; Lung Neoplasms - complications; Male; Middle Aged; Paraneoplastic Syndromes - etiology; Scleroderma, Systemic - etiology
• ISSN: 1776-2588
• DOI: 10.1016/j.rmr.2009.09.005

Despite the known association between scleroderma and cancer, the current systemic sclerosis classifications do not clearly identify paraneoplastic sclerodermiform syndrome or sclerodermiform syndrome secondary to cancer treatments. A 56 year old man was hospitalized for severe Raynaud's phenomenon with bilateral digital necrosis and otherwise good health status. X-rays did not show any subcutaneous calcification. Levels of serum antinuclear antibodies were high but anticentromere and anti-topoisomerase 1 antibodies were negative. Chest X-ray and CT-scan identified an irregular opacity in the right upper lobe with enlarged mediastinal lymph nodes. A diagnosis of bronchial adenocarcinoma was made following mediastinoscopy and the patient was treated with neo-adjuvant chemotherapy and lobectomy. After two cycles of treatment, his skin lesions had almost disappeared. The presence of a sclerodermiform syndrome may suggest the existence of an underlying neoplasm. In this case report, the skin lesions disappeared quickly after antineoplastic treatment. We suggest that sclerodermiform syndrome be included in the systemic sclerosis classification.