Penicillamine induced pseudoxanthoma elasticum with elastosis perforans serpiginosa

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...

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Bibliographic Details
Published in:Indian journal of dermatology, venereology, and leprology venereology, and leprology, 2005-05, Vol.71 (3), p.182
Main Authors: Rath, Namita, Bhardwaj, Amit, Kar, Hemant Kumar, Sharma, P K, Bharadwaj, Meenakhshi, Bharija, S C
Format: Article
Language:English
Subjects:
Adult
Biopsy, Needle
Case studies
Cryotherapy - methods
Diagnosis
Dose-Response Relationship, Drug
Drug therapy
Elastic Tissue - pathology
Follow-Up Studies
Hepatolenticular Degeneration - diagnosis
Hepatolenticular Degeneration - drug therapy
Humans
Immunohistochemistry
Male
Penicillamine
Penicillamine - adverse effects
Penicillamine - therapeutic use
Pseudoxanthoma elasticum
Pseudoxanthoma Elasticum - chemically induced
Pseudoxanthoma Elasticum - pathology
Pseudoxanthoma Elasticum - therapy
Severity of Illness Index
Treatment Outcome
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Summary: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.
ISSN:0378-6323
1998-3611
DOI:10.4103/0378-6323.16234