ized by loose and redundant skin Biopsy resultsare positive for a redu - PDF document

1 114 ized by loose and redundant skin. Bi
114 ized by loose and redundant skin. Biopsy resultsare positive for a reduction in or an absence ofelastic fibers in the dermis. Cutis laxa isacquired or congenital. The acquired form iseither a generalized insidious form (type I) or aform associated with prior inflammation (type II).Cardiovascular, pulmonary, gastrointestinal, andurologic complications may occur. In the past,dyscrasia. We report on a characteristic case ofcutis laxa to alert clinicians to this uncommonbeginning in adulthood (type I). Development of angeneralized cutis laxa. Type II cutis laxa (Marshallloss of elasticity. Acquired Cutis Laxa Associated WithAnjeli Gupta, BS; Thomas N. Helm, MD Ms. Gupta is a medical student at the State University of New York atBuffalo School of Medicine. Dr. Helm is Clinical Associate Professorof Dermatology at the State University of New York at Buffalo. Upon completion of this activity, dermatologists and general practitioners should be able to:1. Recognize the clinical features of cutis laxa.This article has been peer reviewed andapproved by Michael Fisher, MD, Professor ofMedicine, Albert Einstein College of Medicine.Review date: January 2002.in accordance with the E

2 ssential Areas and Policiesof the Accred
ssential Areas and Policiesof the Accreditation Council for Continuing MedicalEducation through the joint sponsorshipof AlbertHealthCom, Inc. The Albert Einstein College ofMedicine is accredited by the ACCME to provideAlbert Einstein College of Medicine designatesin category 1 credit toward the AMA PhysicianÕsRecognition Award. Each physician should claimonly those hours of credit that he/she actually spentin the educational activity.This activity has been planned and produced inaccordance with ACCME Essentials. Cutis Laxa Associated With Multiple MyelomaVOLUME 69, FEBRUARY 2002der, rectal prolapse, pulmonary manifestations (eg,hernias, and vascular abnormalities. Cardiomegaly,aortic dilatation may occur. Uncommon associa-cillamine therapy, and a reaction to penicillin orisoniazid therapy. Middermal elastolysis resultsA 62-year-old woman with a history of multipletherapy, and the patient improved markedly. She Figure 1.Figure 2.Loose redundantskin on the back in areas thathave not received significantsun exposure.Figure not available online Cutis Laxa Associated With Multiple Myelomastimulating factor, thalidomide, and occasional dex-amethasone pulse therapy. At presentat

3 ion, she wasthalidomide 100 mg daily, wi
ion, she wasthalidomide 100 mg daily, with the thalidomidegradually being increased to a total of 400 mg daily.the patientÕs brother and father. Evident on physi- Figure 3.Unremarkable Figure 4.taken from the back werefibers but negative for otherabnormalities (Verhoeff Cutis Laxa Associated With Multiple MyelomaVOLUME 69, FEBRUARY 2002(3340 mg/dL; reference range, 534ÐOur patientÕs skin condition coin- Recommended Algorithm for Evaluation of Patients Suspected of Having Acquired Cutis Laxa EvaluationFindingsBiopsy taken from lax-skin siteElastic fibers reduced or absentSystemic involvementDiverticula of gastrointestinal tract and bladder, ectal prolapse, pulmonary manifestations (eg, emphysema, pulmonary fibrosis), inguinal or hiatal hernias, vascular and cardiovascular abnormalitiesPrior inflammationUrticarial lesions, papular erythemaradiation exposureSkin laxitySerum copper levelDecrease corresponds to abnormal lysyl oxidase activity and abnormal elastin synthesisAmyloidosisApple-green birefringence; deposition of Congo-red Serum elastase inhibitor levelDecreased -antitrypsin Determine if patient has bone pain (pain in back or ribs is most common)erform complete bloo

4 d cell count for serum calcium level, er
d cell count for serum calcium level, erythrocyte sedimentation rate, and presence of anemiaerform serum protein electrophoresis, immunofixation electrophoresiserform bone radiography; perform urine analysis for Bence Jones proteins erform blood smear test for rouleau formation Cutis Laxa Associated With Multiple Myelomais thought to be a factor.(MMP) activity.messenger RNA activity. MMP1 and MMP3metabolism. Lysyl oxidase is a copper-dependentpatientÕs serum copper level was normal).and subsequent elastic fiber degradation. We sus-is outlined in the Table. Although the exactmultiple myeloma is unclear, clinicians should be1.Koch SE, Williams ML. Acquired cutis laxa: case report2.Bouloc A, Godeau G, Zeller J, et al. Increased fibroblast elas-3.Nikko A, Dunnigan M, Black A, et al. Acquired cutis laxa4.Yoneda K, Kanoh T, Nomura S, et al. Elastolytic cuta-5.Ting HC, Foo MH, Wang F. Acquired cutis laxa associated6.Scott MA, Kauh YC, Lunscombe HA. Acquired cutis7.Cho SY, Maguire RF. Multiple myeloma associated with8.Martin L, Requera L, Yus ES, et al. Acral localized9.McCarty MJ, Davidson JM, Cardone JS, et al. Cutis laxa10.Hatamochi A, Kuroda K, Shinkai H, et al. Regulation of

5 11.Holbrook KA, Byers PH. Structural abn
11.Holbrook KA, Byers PH. Structural abnormalities in theHatamochi A, Wada T, Takeda K, et al. Collagen13.Uitto J, Ryhanen L, Abraham P, et al. Elastin in diseases.14.Grassegger A, Romani N, Fritsch P, et al. Immunoglobu-The opinions expressed herein are those of the authors and do not necessarily represent the views of the sponsor or its publisher. Please review complete prescribinginformation of specific drugs or combination of drugs, including indications, contraindications, warnings, and adverse effects before administeringpharACULTYDISCLOSUREThe Faculty Disclosure Policy of the College of Medicine requires that faculty participating in a CME activity disclose to the audience any relationship with a pharma-company that might pose a potential, apparent, or real conflict of interest with regard to their contribution to the program. It is required by theAccreditation Council for Continuing Medical Education that each author of a CME article disclose to the participants any discucial product or device or an investigational use not yet approved by the Food and Drug Administration.Ms. Gupta and Dr. Helm report no conflict of interest. Dr. Fisher reports no conflict of inter