Study design: Long term prospective non-randomised trial to compare therapeutic effectiveness of corticosteroids (CS) and CS plus D-penicillamine (D-pen) in patients with severe eosionophilic fasciiti.

Results: Coadministration of CS and D-pen decreased the affected body surface area from 29 to 8.9 % in average, whereas CS alone lead to a decrease from 28 to 22.83 %. Clinical improvement was observed in all CS+D-pen treated patients compared to 33.3% in CS alone patients.

Conclusions: The results recommend that addition of an antifibrotic agent like D-pen to initial treatment with CS improves the outcome of the of severe eosiniphilic fasciitis.