Calciphylaxis: Risk Factors, Diagnosis, and Treatment Nigwekar, Sagar U., MD, MMSc; Kroshinsky, Daniela, MD, MPH; Nazarian, Rosalynn M., MD ...
American journal of kidney diseases,
07/2015, Letnik:
66, Številka:
1
Journal Article
Recenzirano
Odprti dostop
Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly ...seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function. In this review, we discuss the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and nonuremic calciphylaxis. High-quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence and poorly understood pathogenesis and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by a multidisciplinary team for patients with calciphylaxis.
The yield of skin biopsies in the evaluation of intravascular lymphoma (IVL) is largely unknown in Western patients. Most data supporting this test come from Asian populations, in which both ...prevalence and disease presentation seem to differ.
To determine the yield and diagnostic properties of skin biopsy in the evaluation of IVL.
We reviewed skin biopsy pathology reports of 50 patients being evaluated for IVL to calculate the diagnostic yield of this test. An additional 6 patients, who underwent skin biopsies after the diagnosis of IVL was made by other means, were included to calculate the sensitivity and specificity of our index test.
Skin biopsy samples were positive for 5 of the 50 patients being investigated for IVL. Sensitivity was 50% and specificity was 100%.
Only pathology reports containing IVL as an indication for the biopsy were retrieved. This might have excluded patients in whom the disease was considered but was not deemed likely enough to be listed as the indication for the test, inflating our estimative of skin biopsy yield.
A relatively high diagnostic yield was found in the evaluation of IVL among patients with a diverse presentation in a Western hospital.
Calciphylaxis: Part I. Diagnosis and pathology Rick, Jonathan; Strowd, Lindsay; Pasieka, Helena B. ...
Journal of the American Academy of Dermatology,
20/May , Letnik:
86, Številka:
5
Journal Article
Recenzirano
Calciphylaxis is an uncommon but devastating disorder characterized by vascular calcification and subsequent cutaneous tissue necrosis. This results in exquisitely painful and slow healing wounds ...that portend exceptionally high morbidity and mortality. The diagnosis of this condition can be complicated because there are no conclusive serologic, radiographic or visual signs that this disease is manifesting. The differential of tissue necrosis is broad, and identifying calciphylaxis requires an adroit understanding of the risk factors and physical signs that should raise suspicion of this condition. Reviews on this subject are uncommon and lack directed commentary from disease experts on the best diagnostic approach for patients suffering from this disease. The goal of this article is to update practicing dermatologists on the current standard of care for calciphylaxis.
Clinicians play a critical role in recognizing, initiating, and adopting innovative solutions to clinical problems. Increasing clinician involvement in problem-based innovation will help identify and ...solve unmet medical needs. The overall objective of our program was to increase clinician involvement in problem-based innovation. We pioneered and piloted the “Magic Wand” Initiative (MWI) at Massachusetts General Hospital Department of Dermatology, by inviting clinical faculty to voluntarily participate in problem-driven innovation. The primary outcome was the number of unmet clinical needs identified and pursued by clinicians, who were ‘activated’ to initiate problem-based innovation
.
Other objectives were to enhance clinician-to-clinician dialogue and to develop specific strategic framework for clinician-led, problem-driven research. This pilot MWI was started in 2013 with an announcement at dermatology faculty meeting inviting all clinical faculty to participate on volunteer basis. Academic dermatologists were the main participants in this program. They also contacted, collaborated and worked with research faculty, industry experts and lawyers. Out of 30 unmet needs identified by clinicians participating in MWI, eight are actively being pursued by clinicians. Three of those cases presented here have achieved publications, grant funding, prototype devices and product for patient use. In conclusion, MWI is an innovative approach that educates and equips clinician to identify and solve problems and engages them as leaders in their healthcare ecosystem. MWI has achieved concrete measurable success, affirming that if clinicians are empowered and supported to identify and solve existing unmet medical problems, new and innovative solutions can be invented to improve patient care.
Calciphylaxis: Treatment and outlook—CME part II Rick, Jonathan; Rrapi, Renajd; Chand, Sidharth ...
Journal of the American Academy of Dermatology,
20/May , Letnik:
86, Številka:
5
Journal Article
Recenzirano
Calciphylaxis is a rare and devastating condition with important systemic ramifications. This second-part of our CME aims to educate the practicing dermatologist on the current standard of care once ...a diagnosis of calciphylaxis is confirmed or highly suspected. The key pathologic findings, as well as the role and limitations of biopsy, are reviewed. We aim to guide readers through the complex hospitalization and posthospitalization management of these medically vulnerable patients. Collaboration with other specialists will be discussed. Experimental and developing treatments are discussed, and the outlook of the condition is reported.
Background Given its nonspecific physical examination findings, accurately distinguishing cellulitis from a cellulitis mimicker (pseudocellulitis) is challenging. Objective We sought to investigate ...the national incidence of cellulitis misdiagnosis among inpatients. Methods We conducted a retrospective review of inpatient dermatology consultations at Massachusetts General Hospital, University of Alabama at Birmingham Medical Center, University of California Los Angeles Medical Center, and University of California San Francisco Medical Center in 2008. All consults requested for the evaluation of cellulitis were included. The primary outcomes were determining the incidence of cellulitis misdiagnosis, evaluating the prevalence of associated risk factors, and identifying common pseudocellulitides. Results Of the 1430 inpatient dermatology consultations conducted in 2008, 74 (5.17%) were requested for the evaluation of cellulitis. In all, 55 (74.32%) patients evaluated for cellulitis were given a diagnosis of pseudocellulitis. There was no statistically significant difference in the rate of misdiagnosis across institutions ( P = .12). Patient demographics and associated risk factor prevalence did not statistically vary in patients given a diagnosis of cellulitis versus those with pseudocellulitis ( P > .05). Limitations This study was unable to evaluate all patients admitted with cellulitis and was conducted at tertiary care centers, which may affect the generalizability of the results. Conclusions Cellulitis is commonly misdiagnosed in the inpatient setting. Involving dermatologists may improve diagnostic accuracy and decrease unnecessary antibiotic use.
Calciphylaxis is a rare and life-threatening disease that classically manifests with painful skin lesions. It occurs mainly in patients with end-stage renal disease (ESRD) treated with dialysis, has ...poor outcomes, and has no FDA-approved treatment. Our cohort study aims to examine the clinical and pathological features of calciphylaxis and investigates the correlation between cutaneous clinical manifestations and histopathological findings. Data from 70 calciphylaxis patients who were evaluated at the Massachusetts General Hospital between January 2014 and April 2018 were collected from the institutional electronic database. The median age was 58 years (interquartile range IQR: 49-69 years), 60% were women, and 73% were of white race. Most (74%) patients reported severe pain at the time of calciphylaxis diagnosis with a median pain intensity score of 8/10 (IQR: 6-10) on a 0-10 pain scale. The median time from symptom onset to clinical diagnosis was 9 weeks (IQR: 6-16 weeks). The majority (87%) of patients presented with open necrotic wounds (advanced stage lesion) at the time of diagnosis. Common cutaneous clinical features included ulceration (79%), induration (57%), and erythema (41%), while common pathological features included cutaneous microvascular calcification (86%) and necrosis (73%). The presence of fibrin thrombi in skin biopsies was associated with pain severity (p = 0.04). The stage of a skin lesion positively correlated with the presence of necrosis on histological analyses (p = 0.02). These findings have implications for improving understanding of calciphylaxis origins and for developing novel treatments.
We describe a case of mpox characterized by a circularly distributed facial rash but no identified risk factors. Fomite transmission of monkeypox virus from contaminated linen at a massage spa was ...suspected. Clinicians should consider mpox in patients with consistent clinical syndromes, even in the absence of epidemiologic risk factors.
Background Topical immunotherapy using potent contact allergens, such as squaric acid dibutylester (SADBE), can be used to treat alopecia areata. However, there are conflicting reports regarding the ...best protocol for its administration, particularly with regard to the necessity of an eczematous response after the initial sensitization treatment. Objectives We sought to assess the impact of initial sensitization on subsequent hair regrowth. Methods This is a retrospective review of the use of SADBE in the treatment of alopecia areata in 14 patients. Results Eleven of 14 patients underwent initial sensitization before initiating treatment. Three of these patients had an eczematous reaction after 1 attempt, 5 after ≥2 attempts, and 3 failed to develop a clinical reaction. Four of 14 initiated treatment without a clinical reaction or a previous sensitization. Eleven of 14 patients experienced regrowth, 7 with previous sensitization and 4 without. Limitations This observational study is limited by sample size. Conclusions SADBE sensitization regimens and reactions vary widely, and the absence of an initial eczematous reaction to sensitization does not predict a failed response with continued SADBE treatment.
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