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[Elastosis Perforans Serpiginosa and Wilson Disease: A Rare but Predictable Consequence of Long-term Therapy with D-Penicillamine].

Abstract：

:Elastosis perfurans serpiginosa is a rare perforating dermatosis found primarily in adolescents and young adults, characterized by transepidermal elimination of abnormal elastic fibers. The only drug known capable of inducing elastosis perfurans serpiginosa is D-penicillamine. We report the case of a 52 year-old woman with keratotic papules arranged in an annular pattern with central clearing and centrifugal growth, located in the anterior cervical region. The patient was chronically treated with D-penicillamine for Wilson disease. Lesion biopsy showed transepidermal elimination of thickened, eosinophilic, branched, sawtooth-like elastic fibers. The clinical and pathological findings were consistent with elastosis perfurans serpiginosa secondary to D-penicillamine. It is estimated that elastosis perfurans serpiginosa occurs in 1% of patients treated with D-penicillamine. By blocking directly or indirectly the desmosine cross-links between elastin molecules, D-penicillamine leads to the synthesis of abnormal dermal and extracutaneous elastic fibers. Elastosis perfurans serpiginosa may be the first manifestation of a multisystemic degenerative process of elastic connective tissue. :A elastose perfurante serpiginosa é uma dermatose perfurante rara, encontrada sobretudo em adolescentes e adultos jovens, caraterizada pela eliminação transepidérmica de fibras elásticas anómalas. O único fármaco conhecido capaz de induzir elastose perfurante serpiginosa é a D-penicilamina. Descrevemos o caso de uma doente de 52 anos com pápulas queratósicas confluentes com disposição anular e crescimento centrífugo, localizadas na região cervical anterior. A doente estava cronicamente medicada com D-penicilamina, por doença de Wilson. A biópsia lesional revelou eliminação transepidérmica de fibras elásticas com aumento da eosinofilia, espessadas, ramificadas e de aspeto em dentes de serra. Os achados clinicopatológicos foram compatíveis com elastose perfurante serpiginosa secundária à D-penicilamina. Estima-se que a elastose perfurante serpiginosa ocorra em 1% dos doentes medicados com D-penicilamina. Bloqueando direta ou indiretamente as ligações cruzadas de desmosina da elastina, a D-penicilamina leva à síntese de fibras elásticas anómalas dérmicas, mas também extracutâneas. A elastose perfurante serpiginosa pode ser a primeira manifestação de um processo degenerativo multissistémico do tecido conjuntivo elástico.

journal_name

Acta Med Port

journal_title

Acta medica portuguesa

authors

Castro Pinho A,Cardoso JC,Gouveia M,Oliveira H

doi

10.20344/amp.6749

subject

Has Abstract

pub_date

2016-03-01 00:00:00

pages

227-230

issue

eissn

0870-399X

issn

1646-0758

journal_volume

pub_type

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[Elastosis Perforans Serpiginosa and Wilson Disease: A Rare but Predictable Consequence of Long-term Therapy with D-Penicillamine].