nephrogenic systemic fibrosis: is gadolinium the missing piece to the puzzle?

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Page 1: Nephrogenic systemic fibrosis: Is gadolinium the missing piece to the puzzle?

P2004Cutaneous sarcoidosis erupting within multiple colors of tattoos

Leah Belazarian, MD, University of Massachusetts Medical School, Worcester,MA, United States; Mark Scharf, MD, University of Massachusetts Medical School,Worcester, MA, United States; Nikki Levin, MD, University of MassachusettsMedical School, Worcester, MA, United States

Background: Sarcoidosis is a chronic, multisystemic disorder of unknown etiologycharacterized by granulomatous inflammation. Organs that may be involved includelungs, skin, lymph nodes, spleen, eyes, parotid glands, CNS, heart, joints, and bones.Cutaneous manifestations are noted in up to one third of patients and may occur inthe absence of systemic disease. The most common cutaneous findings includeerythema nodosum, dermal plaques, subcutaneous nodules, scar lesions, and lupuspernio.

Observation: A 38-year-old man presented with the history of sudden developmentof asymptomatic papules within several of his tattoos. His tattoos had been acquiredon different occasions over the previous 10 years, with the last completed 1 yearbefore presentation. He denied dyspnea, cough, arthralgias, or vision changes. Onphysical examination, he was noted to have numerous, large, multi-colored,professional tattoos on his upper and lower extremities bilaterally. Firm dermalpapules were scattered throughout many different tattoos and involved severaldifferent colored areas (red, blue, yellow, and black). Biopsy of a papule revealedgranulomatous dermatitis. Special stains for mycobacteria, fungus, and bacteriawere negative. Polariscopic examination was unremarkable as well. Angiotensinconverting enzyme levels were not elevated. Chest radiograph was consistent withthe diagnosis of sarcoidosis, revealing bilateral and mediastinal lymphadenopathy.Based on the clinical, histologic, and radiographic findings, the patient wasdiagnosed with sarcoidosis.

Discussion: This case is unusual in that lesions of sarcoidosis abruptly occurredin tattoos of different ages and within various tattoo ink colors. The propensity ofcutaneous lesions to occur within old scars and tattoos is a well-known phenom-enon in patients with sarcoidosis. Lesions developing in tattoos have been reportedwithin a single pigment or involving multiple colors. Most commonly, these caseshave been associated with hilar lymphadenopathy/pulmonary disease. This phe-nomenon may be the initial presenting sign of disease and should lead the clinicianto pursue a systemic workup.

Commercial support: None identified.

P2005Mediastinal lipomatosis: Two cases

Samira Skali, MD, UHC Ibn Rochd, Casablanca, Morocco; Mouna Jamali, MD, UHCIbn Rochd, Casablanca, Morocco; Kawtar Zouhair, PhD, UHC Ibn Rochd,Casablanca, Morocco; Hakima Benchikhi, PhD, UHC Ibn Rochd, Casablanca,Morocco

Cortico-induced mediastinal lipomatosis is a non-encapsulated panniculus adiposushypertrophy. It is generaly asymptomatic and of underestimated incidence. Wereport 2 cases of exceptional mediastinal lipomatosis, either by localization and ofrevealing symptoms. Case 1: A 46-year-old woman was followed for mixed connectivetissue disease since November 2002. She weighed 90 kg and was 1.60 m in height.Chest pains, dyspnea at rest, wandering edema, supraclavicular hallows/fossa, and jugular veins swelling appeared 2 months following onset of oralcorticotherpy (1 mg/kg/d). Case 2: A 43-year-old woman had a systemic lupus since1995. Oral corticotherapy was administred at 1 mg/kg/d. The patient medicatedherself and was lost for follow-up. She needed a second cure of corticotherapy in2005 for relapse of lupus. Five years later, she was admitted for cushinoid facies,chest pain, and dyspnea at rest. Comment on the two cases: Chest radiographydisclosed widening of all mediastin layers. Chest TDM disclosed layered lipomatosisof all mediastinum but with no associated chest abnormity. Quick corticotherapyreduction up to 10 mg/d and 25 mg/d respectively allowed symptomologyregressive progression. These two observations are of original aspect for beinguncommon form of lipomatosis as to their mediastinal origin and their revealingsigns. Lipomatoses are special complications of oral corticotherapy (1, 2, and 3).They can occur within few weeks (case 1) up to many years (case 2) after treatmentonset. Their physiotherapy is poorly elucidated. Lipomatoses affect different parts ofaxial topography. Mediastinal localization is found in 15% of treated patients. It is lessfrequent compared to orbital and epidural ones. It is often asymptomatic but can beapparent through alarming symptoms difficult to diagnose, such as is in our cases.TDM and MRI are recommended. Regression following corticotherapy stoppage orreduction is progressive.

Commercial support: None identified.

AB134 J AM ACAD DERMATOL

P2006Nephrogenic systemic fibrosis: Is gadolinium the missing piece to thepuzzle?

Lindsey Bennett, MD, University of Wisconsin Madison, Madison, WI, UnitedStates; Andrea Garrett, MD, University of Wisconsin Madison, Madison, WI,United States

New medical disorders arise infrequently, but nephrogenic systemic fibrosis (NSF)is one such entity. First described in 1997, it exclusively affects renal failure patientsresulting in debilitating progressive fibrosis of the skin and systemic organs.Although much work has been done elucidating the histopathologic changes, atrigger has not been detected. Recently, case reports have implicated gadoliniumcontrast agents as a potential etiology, prompting a warning from the FDA in June of2006. We report a similar case and discuss the literature regarding the effects ofgadolinium on tissue and its potential relationship to the known histopathologiccharacteristics of NSF.

Commercial support: None identified.

P2007Glatiramer acetate and lipoatrophy: A report of 6 cases and review of theliterature

Tarek Afifi, MD, University of British Columbia, Vancouver, BC, Canada, LorneHurst, MD, University of Manitoba, Winnipeg, MB, Canada

Introduction: Glatiramer acetate is an immunomodulating agent used in thetreatment of relapsing remitting multiple sclerosis. Although its mechanism ofaction is not fully understood, studies suggest that its antigenic similarity to myelinbasic protein, a component of the myelin sheath, allows it to compete with myelinantigens for T cellebinding and to induce specific Th2 (suppressor) T cells. Whilethe safety profile of this subcutaneously administered medication is generallyconsidered to be excellent, minor dermatologic side effects are not uncommon.Lipoatrophy at the site of drug injection, previously considered a rare adverse event,is increasingly being reported in the literature and our experience suggests that thiscomplication happens more frequently than initially conceived.

Cases: We report 6 additional cases of lipoatrophy occurring at the site of glatirameracetate injection. All patients were seen in the outpatient dermatology clinic at theHealth Sciences Center in Winnipeg between February 2004 and February 2005.

Literature review: Literature was reviewed via PubMed using the search term‘‘lipoatrophy’’ combined with ‘‘glatiramer acetate.’’ Five articles were identified.

Discussion: The mechanism of glatiramer acetate-induced lipoatrophy has not beendefinitively described, but is thought to result from a drug-induced panniculitis.Clinical and histologic evidence of inflammation support this hypothesis anddistinguish this process from the involutional atrophy induced by repetitivemechanical trauma. Unanswered questions requiring formal analysis include theeffect of site rotation and early avoidance of affected areas on lipoatrophydevelopment and progression; the effect of local anti-inflammatory interventionon both lipoatrophy and drug efficacy; and whether or not glatiramer acetate-induced lipoatrophy is permanent or resolves with time. It has been our experiencethat diligent application of a moderately potent topical steroid to involved sites,along with meticulous site rotation, leads to resolution of lesions. This finding is incontrast to previously reported cases in which lipoatrophic lesions were consideredpermanent. Given the significant cosmetic and psychological sequelae of this sideeffect, further investigation is warranted.

Commercial support: None identified.

FEBRUARY 2007