The "lumpy-bumpy" elastic fiber. A marker for long-term administration of penicillamine

A 29-year-old woman with Wilson's disease developed dermolytic skin lesions 2 years after initiation of treatment with penicillamine. Eight years later, still on penicillamine therapy, striae appeared over both of her breasts. Biopsy of involved skin during the 10th year of treatment with penicillamine revealed characteristic lumpy-bumpy alterations of dermal elastic fibers which were not present in the first skin biopsy 8 years previously. Biopsy of a stria showed changes similar to those in the dermolytic skin lesions. Lumpy-bumpy elastic fibers are pathognomonic for penicillamine-induced elastosis. They are easily recognizable with examination by conventional microscopy. Their appearance may serve as a warning of potentially serious, widespread elastic tissue involvement. These abnormal elastic fibers are not only found in the skin, but also in the lungs.