PT  - JOURNAL ARTICLE
AU  - P. Morales
AU  - M.Á. Martinez
AU  - F. Vera
AU  - G. Romero
TI  - Severe lung involvement in systemic scleromyxoedema: a highly unusual finding
AID  - 10.1183/09031936.02.99109302
DP  - 2002 May 01
TA  - European Respiratory Journal
PG  - 976--979
VI  - 19
IP  - 5
4099  - http://erj.ersjournals.com/content/19/5/976.short
4100  - http://erj.ersjournals.com/content/19/5/976.full
SO  - Eur Respir J2002 May 01; 19
AB  - 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.