Ann Dermatol.  2010 Nov;22(4):468-471. 10.5021/ad.2010.22.4.468.

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

• Na SY
• Choi M
• Kim MJ
• Lee JH
• Cho S

• KMID: 2266191
• DOI: http://doi.org/10.5021/ad.2010.22.4.468

Abstract

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.

Keyword

Cutis laxa; Elastosis perforans serpiginosa; Penicillamine; Wilson's disease

MeSH Terms

Adult
Anetoderma
Cutis Laxa
Cystinuria
Elastic Tissue
Hepatolenticular Degeneration
Humans
Korea
Penicillamine
Skin Diseases
Penicillamine
Skin Diseases

Figure

• 

  Fig. 1 Marked loose folds of skin around the elbow.

• 

  Fig. 2 Multiple match head-sized keratotic papules on the annular erythematous base on the neck.

• 

  Fig. 3 A perforating channel from the dermis through the epidermis with a surrounding inflammatory infiltrate and multinucleate giant cells (H&E,×100).

• 

  Fig. 4 (A) Abnormal elastic fibers in the upper dermis showing transepidermal elimination (Verhoeff-van Gieson, ×100). (B) The "bramble bush" appearance of elastic fibers (Verhoeff-van Gieson, ×400).

Reference

1. O'Donnell B, Kelly P, Dervan P, Powell FC. Generalized elastosis perforans serpiginosa in Down's syndrome. Clin Exp Dermatol. 1992. 17:31–33.

2. Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's dermatology in general medicine. 2008. 7th ed. New York: McGraw-Hill;566–567.

3. Breathnach SM. Champion RH, Burton JL, Burns AD, Breathnach SM, editors. Drug reactions. Rook/Wilkinson/Ebling textbook of dermatology. 1998. 6th ed. Oxford: Blackwell Science;3450–3452.

4. Pass F, Goldfischer S, Sternlieb I, Scheinberg IH. Elastosis perforans serpiginosa during penicillamine therapy for Wilson disease. Arch Dermatol. 1973. 108:713–715.

5. Davis W. Wilson's disease and penicillamine-induced anetoderma. Arch Dermatol. 1977. 113:976.

6. Mehta RK, Burrows NP, Payne CM, Mendelsohn SS, Pope FM, Rytina E. Elastosis perforans serpiginosa and associated disorders. Clin Exp Dermatol. 2001. 26:521–524.

7. Amichai B, Rotem A, Metzker A. D-penicillamine-induced elastosis perforans serpiginosa and localized cutis laxa in a patient with Wilson's disease. Isr J Med Sci. 1994. 30:667–669.

8. Poon E, Mason GH, Oh C. Clinical and histological spectrum of elastotic changes induced by penicillamine. Australas J Dermatol. 2002. 43:147–150.

9. Lewis BK, Chern PL, Stone MS. Penicillamine-induced elastosis of the mucosal lip. J Am Acad Dermatol. 2009. 60:700–703.

10. Price RG, Prentice RS. Penicillamine-induced elastosis perforans serpiginosa. Tip of the iceberg? Am J Dermatopathol. 1986. 8:314–320.

11. Bardach H, Gebhart W, Niebauer G. "Lumpy-bumpy" elastic fibers in the skin and lungs of a patient with a penicillamine-induced elastosis perforans serpiginosa. J Cutan Pathol. 1979. 6:243–252.

12. Sahn EE, Maize JC, Garen PD, Mullins SC, Silver RM. D-penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis. Report of a case and review of the literature. J Am Acad Dermatol. 1989. 20:979–988.

13. Mehregan AH. Elastosis perforans serpiginosa: a review of the literature and report of 11 cases. Arch Dermatol. 1968. 97:381–393.

14. Outland JD, Brown TS, Callen JP. Tazarotene is an effective therapy for elastosis perforans serpiginosa. Arch Dermatol. 2002. 138:169–171.

15. Kelly SC, Purcell SM. Imiquimod therapy for elastosis perforans serpiginosa. Arch Dermatol. 2006. 142:829–830.

16. Bécuwe C, Dalle S, Ronger-Savlé S, Skowron F, Balme B, Kanitakis J, et al. Elastosis perforans serpiginosa associated with pseudo-pseudoxanthoma elasticum during treatment of Wilson's disease with penicillamine. Dermatology. 2005. 210:60–63.