TY - JOUR T1 - Severe lung involvement in systemic scleromyxoedema: a highly unusual finding JF - European Respiratory Journal JO - Eur Respir J SP - 976 LP - 979 DO - 10.1183/09031936.02.99109302 VL - 19 IS - 5 AU - P. Morales AU - M.Á. Martinez AU - F. Vera AU - G. Romero Y1 - 2002/05/01 UR - http://erj.ersjournals.com/content/19/5/976.abstract N2 - Scleromyxoedema is a rare systemic disorder characterized by a lichenoid papular rash. Although scleromyxoedema can involve any organ, very few cases of pulmonary involvement have been reported. Moreover, there are no reports in the literature on treatment of this condition, especially with lung transplantation. The authors report a case of scleromyxoedema in a young man with neurological, skin and respiratory involvement, the latter being mainly characterized by pulmonary emphysema. Due to the serious respiratory compromise, and to the stability of the systemic lesions, a bilateral lung transplantation was performed with successful results at 4.5 yrs. In conclusion, lung transplantation may be required in patients with severe respiratory failure caused by scleromyxoedema. ER -