VOLUME 76 JULY 2005 - PDF document

1 VOLUME 76, JULY 2005 Elastosis perforans
VOLUME 76, JULY 2005 Elastosis perforans serpiginosa (EPS) is a rarecomplication of D-penicillamine therapy. EPS hasbeen reported in patients with Wilson disease,cystinuria, and rheumatoid arthritis after manyyears of high-dose therapy. We report a case ofacquired cutis laxa in a patient with cystinuria.Although both EPS and acquired cutis laxa canbe associated with D-penicillamine therapy, fewcases have been reported with overlapping clin-ical presentations, and previously only inpatients with Wilson disease. We review thecharacteristic clinical and histologic features ofEPS and discuss the potential dermatologicmanifestations of D-penicillamine therapy.Case ReportA 59-year-old white woman with cystinuria diagnosedher cystinuria) and was placed on hemodialysis upper arms, and legs bilaterally. She stated this erup-nation of elastin (Figure 3). The Verhoeff-vanGieson stain highlighted elastic fibers with nodularthe dermis (Figure 4). Foreign bodyÐtype giant cellThe patientÕs history and clinical and histologic find-patientÕs request,

2 conservative therapy was startedings to
conservative therapy was startedings to open wound areas. However, her EPS has con-central plug is seen that, if removed, can result in to D-Penicillamine Therapy With Les B. Rosen, MD; Matthew Muellenhoff, DO; Thi T. Tran, DO; Michelle Muhart, MD Accepted for publication May 5, 2004.Dr. Rosen is in private practice, Fort Lauderdale, Florida. Drs. Muellenhoff and Tran are from NOVA Southeastern University/Sun Coast Hospital, Largo, Florida. Dr. Muhart is in private practice, Lake Worth, Florida.The authors report no conflict of interest.Reprints: Matthew Muellenhoff, DO, NOVA Southeastern University/Sun Coast Hospital, Dermatology Residency program, 1559 Indian Rocks Rd, Largo, FL 33770 50CUTIS described as Òlumpy-bumpy,Ó Òsaw-toothed,Ó orby Verhoeff-van Gieson elastic stain, or imaged byelectron microscopy, are distinctive and specific forinduced EPS is unclear. The drug has been shownhigher doses, D-penicillamine also chelates copper,dase, a copper-dependent enzyme required for cross-theory, reports show that experimentally induc

3 edcopper deficiency has led to productio
edcopper deficiency has led to production of struc-foreign bodyÐtype reaction and transepidermal elim-EPS in copper-deficient patients with Menkes syn- Figure 1. acquired cutis laxa. Figure 2. perforans serpiginosa on with acquired cutis laxa.Figure not available online VOLUME 76, JULY 2005 factors besides the effects of D-penicillamine arecribed in cases of Wilson disease and is presumablyin patients receiving treatment for Wilson dis-our knowledge, we report the first case oflenging; only a few treatment options are reported Figure 3. elastosis perforans serpigi-nosa showing epidermalepidermal elimination ofFigure 4. nodular protrusions associ-induced elastosis perforansserpiginosa (Verhoeff-van 52CUTIS D-penicillamine therapy.well-described complication of long-term D-penicillamine therapy. Defined by loose saggingskin with decreased elasticity, acquired cutis laxasyndrome, erythema multiforme, nephrotic syn-in the treatment of Wilson disease, cystinuria, anddrug-induced dermatopathy, EPS, cutis laxa, andtherapy is noted in Tables

4 1 and 2.EPS as a sole entity. Hill et a
1 and 2.EPS as a sole entity. Hill et alreported a 36-year-long-term D-penicillamine therapy for Wilsonwith Wilson disease who received D-penicillamine Table 1. D-Penicillamine Therapy11,12 Adverse ReactionsIncidence, % Cutaneous reactions25Ð50Gastrointestinal symptoms (anorexia, epigastric pain, nausea, vomiting, diarrhea)17 Dysgeusia12 Proteinuria/hematuria6 Thrombocytopenia4 Leukopenia2 able 2. Induction Mechanism12 Induction MechanismsCutaneous ManifestationsInterference with collagen Penicillamine dermatopathy, elastosis perforans serpiginosa, and elastinexcessive wrinkling, acquired cutis laxa, pseudoxanthoma elasticum Acute sensitivity reactionsUrticaria or macular and papular eruptionsAutoimmune mechanismsPemphigus group, bullous pemphigoid, systemic lupus erythematosus, dermatomyositisUnknown mechanismsLichen planus, psoriasiform dermatitis, seborrheic dermatitisÐlike eruption, alopecia, hypertrichosis, nail changes VOLUME 76, JULY 2005sional skin and an artery. The changes found incases with visceral change had Wilson

5 term D-penicillamine therapy. Difficulty
term D-penicillamine therapy. Difficulty remainsConclusionknown consequence of long-term therapy, theclinical presentation is considered rare. With fewpreviously unreported. Additionally, this caseassociated with D-penicillamine therapy.1.Patterson J. The perforating disorders. 2.Price RG, Prentice RSA. Penicillamine-induced elastosis3.Gebhart W, Bardach H. The Òlumpy-bumpyÓ elastic fiber4.Sahn EE, Maize JC, Garen PD, et al. D-penicillamine-5.Rucker RB, Murray J, Riggins RS. Nutritional copperHill VA, Seymour CA, Mortimer PS. Penicillamine-ilsonÕs disease. 7.Amichai B, Rotem A, Metzker A. D-penicillamine-cutis laxa in a patient with WilsonÕs disease. 8.Deguti MM, Mucenic M, Cancado EL. Elastosis per-forans serpiginosa secondary to D-penicillamine treat-ment in a WilsonÕs disease patient. 9.Uitto J, Ringpfeil F, Pulkkinen L. Heritable disorders ofJorizzo JL, Rapini RP, eds. Dermatology.10.Shapiro SD. Matrix metalloproteinase degradation of extra-12.Bialy-Golan A, Brenner S. Penicillamine-induced bullous Elastosis Perforans Serpiginosa