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Eur Respir J 2002; 19:976-979
Copyright ©ERS Journals Ltd 2002

Severe lung involvement in systemic scleromyxoedema: a highly unusual finding

P. Morales1, M.Á. Martinez1, F. Vera1 and G. Romero2

1 Depts of Pneumology and Pathology, La Fe University Hospital, Valencia (The Valencia Lung Transplant Group) and 2 Dept of Dermatology, Hospital Complex, Ciudad Real, Spain

CORRESPONDENCE: P. Morales, Servicio de Neumología, Hospital Universitario La Fe, Avda, Campanar 21, 46009, Valencia, Spain. Fax: 34 963868789. E-mail: pmm01v@nacom.es

Keywords: lung transplantation, pulmonary emphysema, pulmonary scleromyxoedema, systemic scleromyxoedema

Received: December 29, 1999
Accepted September 13, 2001

Abstract

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.






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Copyright © 2002 by the European Respiratory Society.