Penicillamine induced pseudoxanthoma elasticum with elastosis perforans serpiginosa.
نویسندگان
چکیده
Long term D-penicillamine therapy, especially when used to treat Wilson's disease has been shown to cause elastosis perforans serpiginosa, pseudoxanthoma elasticum perforans and other degenerative dermatoses. We report a 23-year-old male patient who presented with multiple firm papules, nodules over the neck, axillae, front of elbows for five years. He was a known case of Wilson's disease on long-term treatment with penicillamine for the past 12 years. The papulonodular lesions were non-tender and some were discrete while others were arranged in a circinate pattern. There was central scarring of the skin within the circinate lesions. In addition, there were several small yellowish papules on both sides of the neck which eventually became confluent to form plaques. Histopathology confirmed the diagnosis of elastosis perforans serpiginosa and pseudoxanthoma elasticum. He was treated with cryotherapy (using liquid nitrogen through cryojet) for former lesions. The lesions showed remarkable improvement after five sittings. Now the patient is under trientine hydrochloride (750 mg twice daily) for Wilson's disease.
منابع مشابه
Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease.
Elastosis perforans serpiginosa (EPS) is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers. Penicillamine, which is one of the clear triggers for EPS, is a heavy metal chelator that is primarily used for disorders such as cystinuria and Wilson's disease. It may cause alterations in the dermal elastic tissue such as pseudo-p...
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ransepidermal elimination is a disorder in which T dermal stimuli profoundly effect the behavior and structure of the overlying epidermis. Briefly, there are two categories of disorders: the first are primary dermatoses and consist of elastosis perforans serpiginosa and reactive perforating collagenosis; the second group originates from alterations of dermal constituents in preexisting disorder...
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Transepidermal elimination (TE) is a mechanism whereby foreign or altered constituents can be removed from the dermis. This process involves unique morphologic alterations of the epidermis, which forms a channel and thereby facilitates extrusion of the desired dermal components. The phenomenon of TE may occur as a primary process characterizing disorders, such as elastosis perforans serpiginosa...
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To the Editor: Elastosis perforans serpiginosa (EPS) is part of different kinds of perforating diseases that are dependent on factors such as idiopathic, reactive, or drug induced. We report here a case of a patient who presented with multiple reddish‐brown keratinized papules coalescing to form serpiginous plaques in the anterior and posterior of the neck, which was found to be typical D‐penic...
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To cite: Menzies S, Kirby B. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2015-212482 DESCRIPTION A 42-year-old woman presented with a 5-year history of multiple enlarging skin lesions. Her medical history was significant for Wilson’s disease, for which she had taken penicillamine for approximately 20 years. She had multiple small erythaematous keratotic papul...
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عنوان ژورنال:
- Indian journal of dermatology, venereology and leprology
دوره 71 3 شماره
صفحات -
تاریخ انتشار 2005