Stanton K. Wesson

Nephrogenic fibrosing dermopathy in pediatric patients

Nephrogenic fibrosing dermopathy (NFD) is a rare and recently recognized sclerosing skin disorder of unknown etiology. Reported cases have occurred in patients with chronic renal failure, with or without renal replacement therapy. All previous cases have been reported in older adult patients. We describe two pediatric patients who recently developed this condition and review the existing literature for NFD. Our patients included an 8-year-old boy on peritoneal dialysis with no prior renal transplant and a 19-year-old boy on hemodialysis with a history of previous failed renal transplants. We speculate that the recent emergence of this condition and occurrence in patients with chronic renal failure suggest an association with some newer pharmacological agent that has recently come into wide use. Since both our patients also had previously experienced large vessel thrombosis, hypercoagulable states may also be implicated.

Mycophenolate mofetil in dermatology

UNLABELLED Mycophenolate mofetil (MMF) is the prodrug of mycophenolic acid (MPA), a medication used to treat psoriasis in the 1970s until side effects and the concern of carcinogenesis led to its discontinuation. The prodrug, MMF, emerged decades later in the transplant field. Dermatologists have since used MMF off-label to treat various inflammatory skin conditions, with most research concentrating on its use in psoriasis, autoimmune blistering disorders, dermatitides, and connective tissue disorders. The appeal of MMF is predicated upon its lymphocyte specificity and consequent decreased toxicity profile. These attributes may make it a preferable treatment option. Its use in the field of dermatology is currently limited by a lack of randomized controlled trials, potential unknown side effects, and cost of treatment. In reviewing both current literature and our own clinic records, MMF appears to be a promising therapeutic option for the treatment of cutaneous inflammatory diseases. LEARNING OBJECTIVE After completing this learning activity, participants should be able to summarize the history and pharmacology of mycophenolate mofetil as an immunosuppressant; recognize its potential role in the treatment of dermatologic conditions, including general dosing guidelines, use in pregnancy and pediatrics, and potential adverse effects; and identify future considerations and developing areas of research regarding the use of mycophenolate mofetil in dermatology.

Gadolinium and nephrogenic fibrosing dermopathy in pediatric patients

Sirs, We previously published in this journal [1] a report of two pediatric patients who developed nephrogenic fibrosing dermopathy (NFD). A recent report in adult dialysis patients highlights a possible association between intravenous administration of gadolinium-based contrast agents and the subsequent development of NFD [2]. In light of this report and the associated FDA advisory in the United States http://www.fda.gov/cder/drug/advisory/ gadolinium_agents.htm), we went back to the records of our two patients and found that both patients had a history of antecedent magnetic resonance angiograms (MRA) with gadolinium contrast. Patient #2 described in our report had a solitary MRA performed in June 2002, approximately 2 months prior to development of the skin lesions that were diagnosed on skin biopsy as NFD 1 month later. Thus, in this patient, the temporal association was striking. Patient #1 from our previous report was more complicated. This patient had a very complex history of homocystinemia, protein C deficiency and three prior kidney transplants lost to thrombosis. He underwent 13 separate MRA procedures, all with gadolinium contrast, between April 2000 and August 2004, prior to demise in September 2004. At least nine of the MRAs were performed prior to the NFD diagnosis in January 2003. The gap between the last MRA prior to NFD and the skin induration was approximately 2 months. Thus, in this patient, the temporal association was less striking, though also present. Grobner also pointed that their dialysis patients who had MRAs with gadolinium and developed NFD also had metabolic acidosis [2]. In contrast, the dialysis patients who underwent MRA and did not develop NFD had normal blood pH and bicarbonate values. Both our patients exhibited persistent metabolic acidosis in the months preceding the diagnosis of NFD. We have previously speculated about the temporal association of sevelamer hydrochloride use and the emergence of NFD [1]. Metabolic acidosis is a known complication of sevelamer use [3]. Both of our patients, as we previously reported, had received sevelamer therapy. It is possible that multiple risk factors, such as the use of gadolinium in a renal failure patient with concomitant acidosis due to sevelamer use, may be involved in the causation of NFD.

Nonbullous neutrophilic dermatosis: Sweet’s syndrome, neonatal lupus erythematosus, or both?

We describe a 5-day-old infant who fulfilled the diagnostic criteria for Sweet’s syndrome, and the concurrent histologic and autoantibody features supporting the diagnosis of neonatal lupus. To our knowledge, this is the youngest case of Sweet’s syndrome reported in the literature. Importantly, our findings further support the hypothesis that lupus erythematosus should be considered in the differential diagnosis of a nonbullous neutrophilic dermatosis, as it may represent the initial manifestation of the disease.

Pyoderma gangrenosum masquerading as necrotizing fasciitis

Necrotizing fasciitis is a fulminant advancing soft tissue infection characterized by widespread fascial necrosis, which can result in significant morbidity and even death. This condition requires prompt diagnosis and aggressive management with extensive surgical debridement and appropriate antibiotic coverage. Pyoderma gangrenosum, in contrast, is a noninfectious inflammatory condition of the skin that typically does not require surgical management. Both conditions can present with extensive ulceration and tissue necrosis, and close clinical-pathologic correlation is required to make the appropriate diagnosis. We present a case in which the diagnosis of pyoderma gangrenosum was initially elusive and serves to illuminate the diagnostic pitfalls in dealing with these entities.

Malar rash caused by metal allergy in a patient with systemic lupus erythematosus

Background A 61-year-old woman with an 8-year history of systemic lupus erythematosus presented with a non-pruritic, erythematous, malar rash. Previously, she had tested positive for antinuclear antibody and autoantibodies to double-stranded DNA and Ro/SSA, and had an elevated erythrocyte sedimentation rate. She wore eyeglasses with metal frames and had recently gained weight, which caused the eyeglasses to have increased contact area with her face.Investigations Physical examination, autoantibody testing, measurement of complement C3 and C4 levels, measurement of erythrocyte sedimentation rate, hypersensitivity patch testing, dimethylglyoxime test of the patients eyeglass frames.Diagnosis Delayed-type hypersensitivity reaction to nickel and possibly to cobalt dichloride in the patients eyeglass frames, which caused a malar rash that mimicked acute cutaneous lupus erythematosus.Management The rash resolved completely with contact avoidance with the eyeglass frames.

Blastomycosis-like pyoderma: response to systemic retinoid therapy.

Blastomycosis‐like pyoderma is a proliferative tissue response with multifactorial etiologies that may mimic other inflammatory and neoplastic skin disorders. It often occurs on sun damaged skin of immunocompromised patients. We report a case of blastomycosis‐like pyoderma in a patient with underlying metastatic medullary carcinoma of the thyroid receiving treatment with sorafenib (a multikinase inhibitor). Prior treatments were unsuccessful. Our treatment with oral acitretin resulted in significant improvement.

The Dermatologist and Social Media: The Challenges of Friending and Tweeting

The Internet allows student doctors and physicians to rapidly communicate and share information with millions of people. Those who participate in social networking must be aware of the professional pitfalls and legal challenges of social networks on the Internet, and how to honor the moral obligations of the patient-doctor relationship when using these sites. The authors discuss two ethical scenarios that could arise when a dermatologist uses Web 2.0 online social networking websites such as Facebook and Twitter. The authors provide firm recommendations about what is considered professional and unprofessional online content and how to monitor and maintain a professional online presence.

The Mentor-Mentee Relationship: The Devil Is in the Details

Mentoring is a well-established means of professional development in medicine as well as other career fields. Good mentoring is a positive developmental process that can benefit students and doctors at all career stages. Its success is often dependent on the efforts applied by the mentee, as well as the mentor, and good communication between the two. A mentor acts as a teacher and role model who encourages the growth and independent thinking of the mentee, with the ultimate goal to facilitate the mentee’s professional advancement. The relationship formed between the two is dynamic, complex and can either be mutually-beneficial or destructive depending on several factors. Difficulties that may be encountered in the mentorship can be compared to those that occur in any caring relationship. Through the discussion of three cases, this chapter reviews key definitions, stages, and roles within the mentoring relationship that provide insight into possible ethical issues or problems that may arise. Potential conflicts usually stem from poor implementation of mentoring and thus better understanding of the process may result in improved outcomes for both the mentor and mentee. The chapter also provides support for the continued use of mentoring in the professional and social development of the students, residents, and faculty involved in the process.

Rapamycin for refractory discoid lupus erythematosus

Generalized discoid lupus erythematosus can pose a therapeutic challenge for dermatologists. Current treatment emphasizes photoprotection, topical and systemic steroids, and steroid‐sparing immunosuppressive agents if necessary. Rapamycin, also known as sirolimus, selectively inhibits mammalian target of rapamycin, a regulatory kinase responsible for multiple signal transduction pathways. Mammalian target of rapamycin inhibition reduces cell division, lymphocyte proliferation, cytokine release, and downstream pathways unique from other classes of immunomodulatory drugs. Herein, we present a case of generalized discoid lupus erythematosus resistant to topical steroids, prednisone, azathioprine, mycophenolate mofetil, hydroxychloroquine, and thalidomide. The addition of rapamycin led to a positive treatment response within 6 weeks, with good tolerance of the medication and no adverse effects. The current literature supporting the use of rapamycin in the treatment of autoimmune connective tissue diseases is also briefly reviewed. For patients with severe or generalized discoid lupus erythematosus refractory to conventional treatment, rapamycin may be a useful therapeutic consideration.