Elastosis perforans serpiginosa during penicillamine therapy for Wilson disease

Pass, F.

doi:10.1001/archderm.108.5.713

Cited by 28 publications

(16 citation statements)

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“…EPS surely represents one aspect of the skin manifestations of our case, but does not include all of them. Clinical manifestations on the neck, axillae, and groin were mostly identical to those in previously reported cases (24)(25)(26)(27)(31)(32)(33). It is, however, difficult to clearly differentiate cutis hyperelastica, crinkling of skin of face and neck, cutis laxa, anetodermia, and pseudoxanthoma elasticum-like skin changes, because these entities may have a considerable overlap or may represent only minor variations of the same clinical entity.…”

Section: Discussionsupporting

confidence: 70%

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

Iozumi

Nakagawa

Tamaki

1997

The Journal of Dermatology

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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.

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Section: Discussionsupporting

confidence: 70%

“…Various skin manifestations due to penicillamine have been reported and various names applied (21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34). Based upon the estimated pathogenic background and the clinical similarities, we collected the penicillamine-induced dermatoses into three categories as shown in the Table 1.…”

Section: Discussionmentioning

confidence: 99%

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

Iozumi

Nakagawa

Tamaki

1997

The Journal of Dermatology

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“…3 In 1973, Pass et al first showed a series of case reports describing EPS as a sign of dermal elastosis associated with longterm DPA treatment. 5 EPS is a reactive perforating cutaneous disorder resulting from the elimination of abnormal elastic fibres from the upper dermis through the epidermis. 2,3,6 EPS may be idiopathic, associated with several diseases or DPA-induced.…”

Section: Commentmentioning

confidence: 99%

Detection of D‐penicillamine in skin lesions in a case of dermal elastosis after a previous long‐term treatment for Wilson's disease

Neri

Gurioli

Raggi

et al. 2014

Acad Dermatol Venereol

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“…Subsequently, seven additional cases occuring in similar situations have been reported. [15][16][17][18][19] The remaining patient developed EPS following penicillamine treatment for cystinuria (congenital defect of homocystine synthetase). *' Clinically, penicillamine-induced EPS appears similar to primary idiopathic EPS with annular or circinate, keratotic, umbilicated papules on the lower face, sides of neck, axillae, and antecubital fossae; however, one patient had a discoid, firm-bordered penile lesion with a central depressed area within the inner margin of the hyperkeratotic ring.…”

Section: Histologymentioning

confidence: 99%