Marc E. Grossman

Incidence of Nephrogenic Systemic Fibrosis at Two Large Medical Centers

PURPOSE To determine the incidence and associated risk factors of nephrogenic systemic fibrosis (NSF) in patients who undergo gadolinium-based contrast agent (GBCA)-enhanced magnetic resonance (MR) imaging. MATERIALS AND METHODS Institutional review board approval was obtained for retrospective review of the medical records from two hospitals to identify all cases of biopsy-confirmed NSF and all patients administered a GBCA from January 1, 1997, to June 30, 2007. Informed patient consent was not required. The incidence of NSF was calculated for patients who received a standard dose of GBCA, patients who received a high dose, and subgroups of patients with renal impairment. RESULTS Fifteen patients developed NSF after gadolinium-enhanced MR imaging. All of them had an estimated glomerular filtration rate (eGFR) lower than 30 mL/min, and 11 had acute renal failure or acute deterioration of chronic renal failure. The incidence of NSF after gadolinium-enhanced MR imaging without screening for renal function was zero of 74,124 patients with the standard dose of GBCA and 15 (0.17%) of 8997 patients with the high dose (P < .001). The NSF incidence associated with a high dose of GBCA increased to 0.4% in patients in a chronic hemodialysis program and to 8.8% in those who had an eGFR lower than 15 mL/min but were not undergoing hemodialysis (P < .001). The NSF incidence in the patients with acute renal failure who received a high dose when their creatinine level was increasing was 19% (11 of 58 patients) when hemodialysis was delayed for longer than 2 days. More patients with NSF had proinflammatory events, and compared with patients without NSF, these patients had lower pH, younger age, lower eGFR, elevated serum phosphorus levels, and a longer delay between GBCA injection and hemodialysis. CONCLUSION For patients with an eGFR lower than 15 mL/min, hemodialysis helped to prevent NSF. For patients with an eGFR lower than 30 mL/min who received a high dose of GBCA, acute renal failure, delayed hemodialysis after contrast agent injection, proinflammatory events, and hyperphosphatemia were associated with increased risk of NSF.

Nephrogenic Systemic Fibrosis and Its Impact on Abdominal Imaging

The objective of this article is to review the current knowledge about nephrogenic systemic fibrosis (NSF) and how to prevent it. More than 300 cases of NSF in patients with severe chronic renal insufficiency or acute renal failure or in patients undergoing dialysis have been reported in the peer-reviewed literature, with an overwhelming majority occurring within weeks to months after injection of a gadolinium-based contrast agent (GBCA). Because administration of a high dose of a GBCA is a primary risk factor and because most high-dose magnetic resonance (MR) imaging applications involve abdominal imaging (eg, liver and abdominal MR angiography), NSF cases have been associated with abdominal MR imaging. Additional major risk factors for developing NSF include proinflammatory conditions, failure to perform dialysis promptly after GBCA administration, use of nonionic linear contrast agents, hyperphosphatemia, and younger age. Recent recommendations to use GBCAs with caution in patients with acute renal failure, patients receiving dialysis, or patients with an estimated glomerular filtration rate of less than 30 mL/min have resulted in virtually no new NSF cases being reported with onset in 2008 or 2009 in spite of a high level of awareness about this entity. In conclusion, NSF has been virtually eliminated by using caution in administering GBCAs to patients known to have severe or acute renal failure. In these patients, avoid high doses; and for patients undergoing dialysis, schedule MR imaging to occur just before a dialysis session to ensure rapid elimination of gadolinium.

Sweet's syndrome and pregnancy

Abstract We describe a pregnant woman with Sweets syndrome (acute febrile neutrophilic dermatosis) and review the literature.

Chronic hyperkeratotic herpes zoster and human immunodeficiency virus infection

A patient with human immunodeficiency virus infection had hyperkeratotic papules in the T 11 and T 12 dermatomes in which she previously had papulovesicular herpes zoster. Findings of a biopsy specimen and viral culture of these papules subsequently revealed varicella-zoster that eventually responded to prolonged high-dose acyclovir therapy and debridement. A review of reported cases of hyperkeratotic varicella-zoster infections is presented, in addition to our recommendations for the treatment of varicella-zoster infection in patients who have acquired immunodeficiency syndrome.

Becker's nevus and an accessory scrotum

A case of Beckers nevus occurring in association with an accessory scrotum is described. Recent studies have shown the presence of androgen receptors in Beckers nevus in concentrations comparable to those in genital skin. We postulate that the simultaneous occurrence of both these developmental anomalies was manifested because of androgen sensitivity and stimulation.

Penicillamine-associated pemphigus: is it really pemphigus?

Penicillamine-associated bullous eruptions share with spontaneously occurring pemphigus intraepidermal acantholysis, epidermal intercellular deposition of immunoglobulin, and circulating serum antibody against the intercellular regions of the epidermis. We report the case of a penicillamine-associated bullous eruption in which there were some of the histologic features of pemphigus, but none of the immunofluorescent features. Instead, the immunofluorescent findings of bullous pemphigoid were demonstrated. Review of the literature reveals that clinical and histologic features of penicillamine-associated bullous eruptions differ in important respects from those of spontaneously occurring pemphigus. Our report adds immunologic data to evidence that the penicillamine-associated bullous eruptions may not be the same disease as spontaneously occurring pemphigus.

Periumbilical parasitic thumbprint purpura: Strongyloides hyperinfection syndrome acquired from a cadaveric renal transplant

J.A. Weiser, B.E. Scully, W.A. Bulman, S. Husain, M.E. Grossman. Periumbilical parasitic thumbprint purpura: Strongyloides hyperinfection syndrome acquired from a cadaveric renal transplant.
Transpl Infect Dis 2011: 13: 58–62. All rights reserved

Calcium may preferentially deposit in areas of elastic tissue damage

BACKGROUND Cutaneous calcification is an acquired disorder whereby insoluble, amorphous calcium salts deposit in the skin. Classically, cutaneous calcification is categorized as metastatic, dystrophic, idiopathic, or iatrogenic. OBJECTIVE The purpose of this study was to further elucidate the underlying pathogenic mechanism for cutaneous calcification. METHODS Three cases of cutaneous calcification, including clinical characteristics and associated histopathology, were reviewed. Previous reports of cutaneous calcification were searched for in the published literature and included. RESULTS Calcium is distributed within areas of underlying tissue damage (ie, locus minoris resistentiae), and in our cases, occurred specifically at sites of chronic actinic damage and intravenous extravasation tissue injury. LIMITATIONS A small number of clinical cases and previously published reports were reviewed. CONCLUSION We hypothesize that cutaneous calcification may preferentially occur at anatomic sites where tissue integrity has been compromised (ie, locus minoris resistentiae). We suggest one potential mechanism: that cutaneous calcification occurs within dermis that contains damaged elastic fibers. Pseudoxanthoma elasticum may serve as a possible genetic disease model for this process.

Lichenoid eruption associated with ethambutol

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Disseminated Nocardia asteroides with pustules

A case of disseminated Nocardia asteroides in a renal transplant recipient with cavitary lung lesions and cutaneous pustules is presented. Disseminated nocardiosis is a rare disease that occurs chiefly in the immunocompromised host and usually begins as a pleuropulmonary infection. Disseminated nocardiosis should be included in the differential diagnosis of pustules in immunosuppressed patients.