Elastosis Perforans Serpiginosa
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Letters Figure. Purpuric Contact Dermatitis in Reaction to Methyl Methacrylate A B A, Mild edema and erythema with superficial desquamation is seen along the first, second, and third digits. B, A prominent purpuric patch located on the distal tip of the first finger was noted on the patient’s left hand. nated glove (4H) appears relatively impermeable to MMA, but 2. Lönnroth EC, Wellendorf H, Ruyter E. Permeability of different types of widespread use is limited by cost and diminished dexterity.2,3 medical protective gloves to acrylic monomers. EurJOralSci. 2003;111(5): 440-446. Allergic contact dermatitis induced by MMA may result in 3. Kanerva L, Estlander T, Jolanki R, Tarvainen K. Occupational allergic contact significant discomfort and is seen frequently after occupa- dermatitis caused by exposure to acrylates during work with dental prostheses. tional exposure among dentists, dental technicians, orthope- Contact Dermatitis. 1993;28(5):268-275. dic surgeons, and other health care workers. Second- and third- 4. Bjorkner B. Plastic materials. In: Frosch PJ, Menne T, Lepoittevin JP, eds. digit fingertips are commonly involved.3 Nail dystrophy and Contact Dermatitis. 4th ed. Berlin, Germany: Springer; 2006:583-621. fingertip paresthesias have also been reported.4 Severe cases 5. Lazarov A, Cordoba M. Purpuric contact dermatitis in patients with allergic of MMA-induced dermatitis can even necessitate profession reaction to textile dyes and resins. J Eur Acad Dermatol Venereol. 2000;14(2): 101-105. changes.3 6. Koch P, Baum HP, John S. Purpuric patch test reaction and venulitis due to Purpuric contact dermatitis has been reported in patients methyl methacrylate in a dental prosthesis. Contact Dermatitis. 1996;34(3): sensitized to agents such as textile dyes, formaldehyde, and 213-215. epoxy resins.5 We report herein the second case, to our knowl- edge, of MMA-induced PCD.6 Although patch testing is con- sidered the diagnostic gold standard for suspected allergic con- Elastosis Perforans Serpiginosa: tact dermatitis, this patient’s strong contact history and rapid, A Case of a Penicillamine-Induced sustained resolution of symptoms with allergen avoidance al- Degenerative Dermatosis lowed for clinical diagnosis. Fingertip purpura may appear alarming to unsuspecting clinicians and may prompt an ex- Report of a Case | A man in his 60s presented in reduced gen- tensive workup. We encourage physicians to consider PCD in eral condition and with asymptomatic brownish-red pap- patients with potential contact exposures. ules organized in multiple arcuate to annular formations on his upper trunk and arms (Figure 1). A prominent cutis laxa Lauren Strazzula, BA and cutis rhomboidalis nuchae were noted. At the time of Shinjita Das, MD presentation, he had been treated for Wilson disease with Vinod E. Nambudiri, MD, MBA daily doses of D-penicillamine (1.0-1.5 g/d) for more than 40 Daniela Kroshinsky, MD, MPH years. Histopathologic analysis revealed channels through the Author Affiliations: Department of Dermatology, Massachusetts General epidermis formed by follicular epithelium (Figure 2A). The Hospital, Boston. infundibula were filled with granular cellular debris, neu- Corresponding Author: Daniela Kroshinsky, MD, MPH, Department of trophils, and corneocytes (Figure 2A and C). The interfol- Dermatology, Massachusetts General Hospital, 50 Staniford St, 200, Boston, licular tissue showed a mixed inflammatory infiltrate. Elas- MA 02114 ([email protected]). tica van Gieson staining demonstrated an accumulation of Published Online: May 21, 2014. doi:10.1001/jamadermatol.2013.8520. altered elastic fibers (Figure 2B) within the upper part of the dermis. Conflict of Interest Disclosures: None reported. From the clinical and histopathologic findings, the diag- 1. America GC. Alike Temporary Crown Bridge and Resin: Material Safety Data Sheets. http://www.gcamerica.com/products/operatory/alike/msds.php. Accessed nosis of elastosis perforans serpiginosa (EPS), caused by long- July1,2013. term ingestion of D-penicillamine, was made. jamadermatology.com JAMA Dermatology July 2014 Volume 150, Number 7 785 Copyright 2014 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Letters Figure 1. Clinical Images of D-Penicillamine–Induced Elastosis Perforans Serpiginosa A B A, Overview, with black frame enclosing the affected area shown in panel B; the abnormal posture of the patient is a symptom of rigid dystonia. B, Close-up image of the framed area from panel A showing prominent cutis laxa and annular confluent patches with a central skin atrophy and a peripheral rim of red papules covered by white-yellow scales (scale bars indicate centimeters). Sun-exposed nuchal skin showed increased elastosis with severe cutis laxa and cutis rhomboidalis nuchae. Figure 2. Histopathologic Images of D-Penicillamine–Induced Elastosis Perforans Serpiginosa A B C A, Transepidermal/follicular channels through the surface epithelium that “lumpy-bumpy” fibers indicating induction by penicillamine (original contain basophilic necrotic material (hematoxylin-eosin, original magnification magnifications ×40 for main panel, ×400 for inset). C, Suppurative folliculitis ×40). B, Elastica van Gieson–stained sections showing accumulation of with transinfundibular elimination of altered elastic fibers (hematoxylin-eosin, abnormal elastic fibers in the upper part of the dermis; inset shows original magnification ×100). Discussion | Wilson disease is a rare autosomal recessive condi- quently cutis laxa has been described as an adverse effect of tion caused by a genetic defect in the copper-transporting ATPase D-penicillamine.4 Morphologic changes of the elastic fibers of ATP7B.1,2 Copper accumulation in the liver and the basal ganglia arteries and pulmonary tissue are a matter of concern. In our of the brain may lead to hepatocerebral degeneration. In our pa- patient, the findings of chest and cardiovascular examina- tient, liver transaminase levels were slightly elevated, and neu- tions were unremarkable. rologic symptoms included ataxia and rigid dystonia. Histopathologically, an acanthotic, hyperkeratotic epider- An effective treatment for Wilson disease is D-penicillamine, mis with a mixed dermal inflammatory infiltrate with few gi- a chelating agent that depletes copper. In addition, successful ant cells is present in EPS. Epidermal invaginations with kera- treatment has also been reported with trientine dihydrochlo- totic plugs at the surface form perforating channels filled with ride, zinc, tetrathiomolybdate, and liver transplantation.2 Long- basophilic material. Atrophy of the skin may be found at the cen- term D-penicillamine therapy can induce EPS by reducing the ter of lesions. Elastic fiber stain shows a thickened and coarse activity of lysyl-oxidase, a copper-dependent enzyme that morphology giving rise to the typical “lumpy-bumpy” picture.5 cross-links dermal elastic fibers, and by formation of com- Our patient had been taking D-penicillamine for more than plexes with precursors of elastic fibers, thus impairing their 40 years, well above the average 10-year interval after which EPS maturation.3 As a result, abnormal elastin aggregates pro- may be acquired. For patients with cutaneous or systemic D- mote a foreign body reaction with subsequent transepider- penicillamine adverse effects, an alternative copper-chelating mal elimination. agent like trientine dihydrochloride may be used.2 To our knowl- Clinically, EPS lesions present with multiple keratotic pap- edge, no elastolytic dermatoses have been associated with tri- ules arranged in arcuate or circinate patterns. Lesions are typi- entine dihydrochloride therapy. Therefore, we recommended cally found on the neck and upper extremities. Much less fre- that the patient shift oral treatment from D-penicillamine to tri- 786 JAMA Dermatology July 2014 Volume 150, Number 7 jamadermatology.com Copyright 2014 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Letters entine dihydrochloride in this interdisciplinary case. For the re- tissue. To our knowledge, as of 2006, there were a total of 86 maining lesions, we offered liquid nitrogen cryotherapy or ab- reported cases of cutaneous Rosai-Dorfman disease (CRDD) in lative laser resurfacing to the patient. Unfortunately, the patient the literature,2,3 with several additional cases reported since was subsequently lost to follow-up. then. Simin Hellriegel, MD Report of a Case | An African American woman in her 50s re- Hans P. Bertsch, MD ported a sudden eruption of dozens of facial papules and nod- Steffen Emmert, MD ules 3 months previously and a 7-month history of a groin Michael P. Schön, MD plaque. She had been treated with cefadroxil, topical clobeta- Holger A. Haenssle, MD sol, oral prednisone, and a short course of oral isotretinoin with- out significant benefit. Author Affiliations: Clinic of Dermatology, Venereology and Allergology, Physical examination revealed approximately 75 pink- University Medical Center, Georg-August-University, Göttingen, Germany domed papules on the cheeks, upper lip, and chin (Figure 1) (Hellriegel, Bertsch, Emmert, Schön, Haenssle). and dozens of red-brown papules becoming confluent on the Corresponding Author: Holger A. Haenssle, MD, Clinic of Dermatology, Venereology and Allergology, University Medical Center, right inguinal and suprapubic skin. Laboratory examination Georg-August-University, Göttingen, Robert-Koch Strasse