Severe lung involvement in systemic scleromyxoedema: a highly unusual finding

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. Eur Respir J 2002; 19: 976-979.

Scleromyxedema treatment: a systematic review and update

“…Case reports have documented clinical improvement in patients treated with topical betamethasone and topical dimethyl sulfoxide, 74 topical and intralesional corticosteroid therapy, topical tacrolimus, 75,76 cyclosporine, 77,78 HCQ, 79 and chemotherapeutic agents including cyclophosphamide, 80‐83 MTX, 84 and 2‐chlorodeoxyadenosine 85 . Retinoids (oral isotretinoin and acitretin) 86,87 have been shown to modulate the glycosaminoglycan synthesis and reduce the mucin production by fibroblasts in SMX, leading to clinical improvement.…”