Elastosis perforans serpiginosa (EPS) is a rare complication of chronic therapy with a high-dose of D-penicillamine (1 g daily for more than 5 years), characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. D-penicillamine (DPA) is a heavy metal chelator primarily used for disorders such as cystinuria and Wilson disease. This therapy can lead to induction of EPS through a still unknown mechanism. We report the follow-up of a D-penicillamine-induced EPS in patient with Wilson disease, which prompted us to switch the therapy with trientine (another metal chelator). After 14 years the cutaneous lesions are still visible; therefore, we conclude that the DPA-induced cutaneous damage is irreversible.
An 18-year follow-up of a case of D-penicillamine-induced Elastosis perforans serpiginosa
Narcisi, A;Rossi, A;
2011-01-01
Abstract
Elastosis perforans serpiginosa (EPS) is a rare complication of chronic therapy with a high-dose of D-penicillamine (1 g daily for more than 5 years), characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. D-penicillamine (DPA) is a heavy metal chelator primarily used for disorders such as cystinuria and Wilson disease. This therapy can lead to induction of EPS through a still unknown mechanism. We report the follow-up of a D-penicillamine-induced EPS in patient with Wilson disease, which prompted us to switch the therapy with trientine (another metal chelator). After 14 years the cutaneous lesions are still visible; therefore, we conclude that the DPA-induced cutaneous damage is irreversible.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
