d-Penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis

Sahn, Eleanor E.; Maize, John C.; Garen, Paul D.; Mullins, Stephen; Silver, Richard M.

doi:10.1016/s0190-9622(89)70123-7

Cited by 36 publications

(16 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…EPS and pseudo-PXE are primarily reported in WD [3, 8], and in few cases in cystinuria [4]which necessitates long-term and high-dose therapy (1–3 g/day). However, it can also occur with low-dose therapy in rheumatoid arthritis [5, 9, 10]. …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Bécuwe¹,

Dalle²,

Ronger-Savlé³

et al. 2005

Dermatology

View full text Add to dashboard Cite

Background: Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA). The first case of EPS induced by DPA was described in 1972 in a patient treated for Wilson’s disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE. Case Report: We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson’s disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation. Discussion: Penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The clinical findings are similar to idiopathic EPS. The neck and upper extremities are most commonly affected but locations on axillae [11], thighs [9], ankles [12]and glans penis [1]have also been reported.…”

Section: Discussionmentioning

confidence: 99%

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

Bécuwe¹,

Dalle²,

Ronger-Savlé³

et al. 2005

Dermatology

View full text Add to dashboard Cite

show abstract

“…In many cases, the trigger for elastogenesis is unknown. D-Penicillamine therapy for Wilson disease, 8,52-58 cystinuria, 59-61 and rheumatoid arthritis 62,63 have been associated with one subtype of EPS.…”

Section: Elastosis Perforans Serpiginosamentioning

confidence: 99%

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

Lewis

Bercovitch

Dill

et al. 2004

Journal of the American Academy of Dermatology

171

178

View full text Add to dashboard Cite

“…The characteristics and specific histological changes of penicillamine-induced elastic fiber damage are thickened elastic bundles with prominent lateral protrusions giving the so-called 'bramble-bush' appearance 11 . In contrast to the calcification of the shortened, curled basophilic elastic fibers in the inherited forms of PXE, calcification of elastic fibers in penicillamine-induced elastosis is considered to be unusual 12 . As was present in this case, a variable degree of granulomatous inflammation is often observed in the dermis 11,12 .…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to the calcification of the shortened, curled basophilic elastic fibers in the inherited forms of PXE, calcification of elastic fibers in penicillamine-induced elastosis is considered to be unusual 12 . As was present in this case, a variable degree of granulomatous inflammation is often observed in the dermis 11,12 . Transepidermal elimination of abnormally large and tortuous eosinophilic 'bramble-bush' elastic fibers is an additional feature of penicillamine-induced EPS 13 .…”

Section: Discussionmentioning

confidence: 99%

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Choi

Kim

et al. 2010

Ann Dermatol

View full text Add to dashboard Cite

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-pseudoxanthoma elasticum, acquired cutis laxa, EPS and anetoderma. Herein we present a case of cutis laxa and EPS in a 34-year-old man who was previously on a long-term, high-dose of penicillamine for Wilson's disease. The combination of EPS and cutis laxa induced by penicillamine has rarely been reported and we report the first such case in Korea. (Ann Dermatol 22(4) 468 ∼471, 2010)

show abstract

d-Penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis

Cited by 36 publications

References 13 publications

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

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

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

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Contact Info

Product

Resources

About