Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter to the Editor - Case Letter
2016:82:4;452-454
doi: 10.4103/0378-6323.178907
PMID: 27279318

Two cases of D-penicillamine-induced elastosis perforans serpiginosa

Jun Liang, Duoqin Wang, Jinhua Xu, Lianjun Chen
 Department of Dermatology, Fudan University, Huashan Hospital, Jing'an, Shanghai 200040, Peoples Republic of China

Correspondence Address:
Lianjun Chen
Department of Dermatology, Fudan University, Huashan Hospital, 12 Wulumuqi Zhong Road, Jing'an District, Shanghai 200040
Peoples Republic of China
How to cite this article:
Liang J, Wang D, Xu J, Chen L. Two cases of D-penicillamine-induced elastosis perforans serpiginosa. Indian J Dermatol Venereol Leprol 2016;82:452-454
Copyright: (C)2016 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Elastosis perforans serpiginosa is a reactive perforating dermatosis in which elastic fibers are extruded through the epidermis. Although D-penicillamine appears to be a clear trigger for elastosis perforans serpiginosa, D-penicillamine-induced disease has been rarely reported.[1] D-penicillamine is a well-known heavy metal chelator, classically used in the treatment of Wilson's disease, rheumatoid arthritis and cystinuria. D-penicillamine-induced skin manifestations can be categorized into three groups: acute hypersensitivity reactions, bullous dermatoses and degenerative dermatoses.[2]

The first patient was a 32-year-old man who had been diagnosed with Wilson's disease at the age of 16 and had been taking D-Penicillamine, 0.75–1.5 g daily since then. He presented to us with a two-year history of slightly itchy, red, keratotic papules, 3–5 mm in diameter, arranged in annular, serpiginous, geographic and other irregular patterns on the neck. The annular lesions showed slight central atrophy and hyperpigmentation [Figure - 1]. He was crippled, and his eyes showed Kayser–Fleischer rings. Serum and urinary copper levels were raised on multiple occasions. A liver biopsy in 2005 had suggested cirrhosis.

Figure 1: Case 1: Red keratotic papules grouped in annular configuration on the neck

Skin biopsy from the raised border of a lesion demonstrated a markedly acanthotic epidermis with a trans-epidermal channel containing thick, coarse elastic fibers. Abundant abnormal, clumped, elastic fibers were present in the reticular dermis. These were confirmed to be elastic fibers on staining with Verhoeff–van Gieson stain [Figure - 2]. The striking feature noted at higher magnification was the presence of multiple serrations and buds arising perpendicularly from the elastic fibers [Figure - 3]. It was not possible to discontinue D-penicillamine because no good alternative for the treatment of Wilson's disease is available in China. He was advised to use tazarotene gel topically which resulted in slow improvement.

The second patient was a 38-year-old man, also a known case of Wilson's disease, on treatment with D-penicillamine, which had commenced when he was 9 years old. He presented to us with slightly itchy, garnet-colored papules coalescing to form serpiginous plaques with raised hyperkeratotic rims and central clearing, atrophy and hypopigmentation [Figure - 4], located on the neck. The rest of the physical examination was normal; there was no Kayser-Fleischer ring and serum and urinary copper levels were also normal. Histopathology [Figure - 5] and [Figure - 6] confirmed the diagnosis of elastosis perforans serpiginosa. He was treated with topical corticosteroids with very slow improvement. Because the small lesion was not troublesome, the patient refused further treatment.

Figure 2: Case 1: Markedly acanthotic epidermis with a trans-epidermal wavy channel (Verhoeff-van Gieson, ×50)
Figure 3: Case 1: Multiple serrations and buds arising perpendicularly from the borders of the elastic fibers (Verhoeff-van Gieson, ×400)
Figure 4: Case 2: Multiple reddish-brown papules coalescing to form serpiginous plaques
Figure 5: Case 2: Destroyed elastic fibers extruded through the epidermis (Hematoxylin and Eosin, ×50)
Figure 6: Case 2: Elastic fibers, mainly of the reticular dermis, are coarser, serrated with saw-tooth like borders and with perpendicular budding from the surface(Verhoeff–van Gieson, ×100)

There are two proposed mechanisms that contribute to the development of D-penicillamine-induced elastopathy. First, the enzyme lysyl oxidase that is required for the cross-linkage of elastic and collagen fibers in the dermis is copper dependent.[3] D-penicillamine chelates the copper cofactor thus indirectly inhibiting lysyl oxidase activity resulting in accumulation of abnormal elastic fibers. Second, D-penicillamine itself may also cause post-translational inhibition of collagen synthesis and lead to defective cross-linking.[4] The clinical features seen in D-penicillamine-induced elastosis perforans serpiginosa are similar to those seen in the other forms of the disease. As illustrated in this report, D-penicillamine-induced elastosis perforans serpiginosa mostly manifests as small, horny, umbilicated papules arranged linearly, circularly or in serpiginous patterns. Lesions commonly involve the face, back and sides of the neck and flexor surface of the upper extremities. Rarely, involvement of the lip and the penis has been reported in the form of hyperpigmented, atrophic, annular plaques with slightly raised borders.[5],[6]

The histology of the D-penicillamine-induced form is special. Compared with other forms of elastosis perforans serpiginosa, there is no elastic fiber hyperplasia in the papillary dermis and a variable degree of granulomatous inflammation is often observed in the dermis. With elastic tissue stains, elastic fibers, mainly in the reticular dermis are coarser, serrated with saw-tooth borders and show perpendicular budding from the surface. These very peculiar changes in the elastic fibers have been termed the “lumpy-bumpy” or “bramble-bush appearance.” [7]

D-penicillamine-induced elastosis perforans serpiginosa is frequently associated with other diseases of the dermal soft tissue such as pseudoxanthoma elasticum and cutis laxa. Moreover, similar changes have also been demonstrated in the elastic tissues of arteries and lungs and therefore, systemic examination is recommended for these patients.[8]

In the treatment of D-penicillamine-induced elastosis perforans serpiginosa, it is preferable to discontinue the use of D-penicillamine. Moderately effective treatments include cryotherapy, oral isotretinoin and tazarotene gel.[9]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Hellriegel S, Bertsch HP, Emmert S, Schön MP, Haenssle HA. Elastosis perforans serpiginosa: A case of a penicillamine-induced degenerative dermatosis. JAMA Dermatol 2014;150:785-7.
[Google Scholar]
2.
Mehta RK, Burrows NP, Payne CM, Mendelsohn SS, Pope FM, Rytina E. Elastosis perforans serpiginosa and associated disorders. Clin Exp Dermatol 2001;26:521-4.
[Google Scholar]
3.
Matsuda I, Pearson T, Holtzman NA. Determination of apoceruloplasmin by radioimmunoassay in nutritional copper deficiency, Menkes' kinky hair syndrome, Wilson's disease, and umbilical cord blood. Pediatr Res 1974;8:821-4.
[Google Scholar]
4.
Nimni ME. Penicillamine and collagen metabolism. Scand J Rheumatol Suppl 1979;(28):71-8.
[Google Scholar]
5.
Lewis BK, Chern PL, Stone MS. Penicillamine-induced elastosis of the mucosal lip. J Am Acad Dermatol 2009;60:700-3.
[Google Scholar]
6.
Roest MA, Ratnavel R. Elastosis perforans serpiginosa of the penis. BJU Int 2003;91:427.
[Google Scholar]
7.
Atzori L, Pinna AL, Pau M, Aste N. D-penicillamine elastosis perforans serpiginosa: Description of two cases and review of the literature. Dermatol Online J 2011;17:3.
[Google Scholar]
8.
Price RG, Prentice RS. Penicillamine-induced elastosis perforans serpiginosa. Tip of the iceberg? Am J Dermatopathol 1986;8:314-20.
[Google Scholar]
9.
Rath N, Bhardwaj A, Kar HK, Sharma PK, Bharadwaj M, Bharija SC. Penicillamine induced pseudoxanthoma elasticum with elastosis perforans serpiginosa. Indian J Dermatol Venereol Leprol 2005;71:182-5.
[Google Scholar]

Fulltext Views
47

PDF downloads
41
Show Sections