Penicillamine‐Induced Degenerative Dermatoses: Report of a Case and Brief Review of Such Dermatoses

Abstract

We describe a case of elastosis perforans serpiginosa with additional findings of degenerative skin changes. A 20-year-old man with hepatolenticular degeneration, under prolonged treatment with D-penicillamine, presented with a circular or serpiginous arrangement of nuchal papules. Histopathologically, transepidermal channels were accompanied by granulomatous reactions, with several giant cells engulfing elastic fibers. In addition to these findings of a typical elastosis perforans serpiginosa, we observed scar-like skin changes inside the circular arrangement of the papules. At the scar-like tissue, we found electron-microscopical evidence of randomly aggregated thin collagen fibers with no tendency toward systemic combined bundle formation, which is a characteristic feature of normal collagen fiber formation. Pseudoxanthoma-elasticum-like changes were observed on his neck. On his axillae and groin, slight skin thickening and wrinkling were detected. The diagnosis of elastosis perforans serpiginosa does not represent all of the manifestations or the pathological background described above. The skin manifestations described here represent not only an elastosis but also a total degenerative dermatosis with overhealed collagenosis. Thus, those dermatoses should be summarized as one entity, penicillamine-induced degenerative dermatosis. After considering the pathogenic background and clinical similarities, we further propose to simplify the penicillamine-induced skin manifestations to three categories: acute sensitivity reactions, bullous dermatoses, and degenerative dermatoses.

Dermatological signs in Wilson’s disease

“…The presence of dermatological findings was not correlated with drug usage. Various skin manifestations due to penicillamine therapy have been reported in WD, such as elastosis perforans serpiginosa, pseudoxanthoma elasticum‐like skin changes, cutis laxa and so on 9,19,20 . None of the present patients had such manifestations.…”

Elastosis perforans serpiginosa Associated with Pseudo-Pseudoxanthoma elasticum during Treatment of Wilson’s Disease with Penicillamine

“…The association of EPS and pseudo-PXE is explained by a similar pathogenesis, but has been reported only rarely [4, 8, 14]. …”

Acquired disorders of elastic tissue: part I. increased elastic tissue and solar elastotic syndromes

“…Several acquired elastic tissue disorders have been reported in association with D-penicillamine therapy; they include elastosis perforans serpiginosa (EPS), cutis laxa, anetoderma, and pseudoxanthoma elasticum (PXE)elike skin changes. 8 Elastin is metabolized by proteolytic enzymes, including serine-type elastases, which are secreted by neutrophils, macrophages, human fibroblasts, and other types of cells, although the role of elastase in the pathogenesis of elastolytic disorders is uncertain. Elastic fibers are also degraded by matrix metalloproteinases (MMPs), of which there are at least 23 different enzymes secreted by a variety of types of cells.…”