To report on the survival and the associations of treatments upon survival of patients with calciphylaxis seen at a single center.
Using the International Classification of Diseases, Ninth Revision ...diagnosis code of 275.49 and the keyword "calciphylaxis" in the dismissal narrative, we retrospectively identified 101 patients with calciphylaxis seen at our institution between January 1, 1999, through September 20, 2014, using a predefined, consensus-developed classification scheme.
The average age of patients was 60 years: 81 (80.2%) were women; 68 (68.0%) were obese; 19 (18.8%) had stage 0 to 2 chronic kidney disease (CKD), 19 (18.9%) had stage 3 or 4 CKD; 63 (62.4%) had stage 5 or 5D (dialysis) CKD. Seventy-five patients died during follow-up. Six-month survival was 57%. Lack of surgical debridement was associated with insignificantly lower 6-month survival (hazard ratio HR=1.99; 95% CI, 0.96-4.15; P=.07) and significantly poorer survival for the entire duration of follow-up (HR=1.98; 95% CI, 1.15-3.41; P=.01), which was most pronounced in stage 5 or 5D CKD (HR=1.91; 95% CI, 1.03-3.56; P=.04). Among patients with stage 5/5D CKD, subtotal parathyroidectomy (performed only in patients with hyperparathyroidism) was associated with better 6-month (HR=0.12; 95% CI, 0.02-0.90; P=.04) and overall survival (HR= 0.37; 95% CI, 0.15-0.87; P=.02).
Calciphylaxis is associated with a high mortality rate. Significantly effective treatments included surgical debridement and subtotal parathyroidectomy in patients with stage 5/5D CKD with hyperparathyroidism. Treatments with tissue-plasminogen activator, sodium thiosulfate, and hyperbaric oxygen therapy were not associated with higher mortality.
To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival.
Study subjects were 101 patients with calciphylaxis seen at ...Mayo Clinic from 1999 to September 2014. Data including thrombophilia profiles were extracted from the medical records of each patient. Survival status was determined using patient registration data and the Social Security Death Index. Survival was estimated using the Kaplan-Meier method, and associations were evaluated using Cox proportional hazards models.
Sixty-four of the 101 patients underwent thrombophilia testing. Of these, a complete test panel was performed in 55 and a partial panel in 9. Severe thrombophilias observed in 60% (33 of 55) of the patients included antiphospholipid antibody syndrome protein C, protein S, or antithrombin deficiencies or combined thrombophilias. Of the 55 patients, severe thrombophilia (85%, 23 of 27) was noted in patients who were not on warfarin at the time of testing (27). Nonsevere thrombophilias included heterozygous factor V Leiden (n=2) and plasminogen deficiency (n=1). For the comparison of survival, patients were divided into 3 treatment categories: Warfarin (n=63), other anticoagulants (n=20), and no anticoagulants (n=18). There was no statistically significant survival difference between treatment groups.
Laboratory testing reveals a strikingly high prevalence of severe thrombophilias in patients with calciphylaxis, underscoring the importance of congenital and acquired thrombotic propensity potentially contributing to the pathogenesis of this disease. These findings may have therapeutic implications; however, to date, survival differences did not vary by therapeutic choice.
Background Although psoriasis is considered to have a dual peak in age of onset, currently no studies exist regarding the incidence of psoriasis in children. Objective The objective of this study was ...to determine the incidence of psoriasis in childhood. Methods A population-based incidence cohort of patients aged younger than 18 years first given the diagnosis of psoriasis between January 1, 1970, and December 31, 1999, was assembled. The complete medical record of each child was reviewed and psoriasis diagnosis was validated by a confirmatory diagnosis in the medical record by a dermatologist or medical record review by a dermatologist. Age- and sex-specific incidence rates were calculated and were age and sex adjusted to 2000 US white population. Results The overall age- and sex-adjusted annual incidence of pediatric psoriasis was 40.8 per 100,000 (95% confidence interval: 36.6-45.1). When psoriasis diagnosis was restricted to dermatologist-confirmed subjects in the medical record, the incidence was 33.2 per 100,000 (95% confidence interval: 29.3-37.0). Incidence of psoriasis in children increased significantly over time from 29.6 per 100,000 in 1970 through 1974 to 62.7 per 100,000 in 1995 through 1999 ( P < .001). Chronic plaque psoriasis was the most common type (73.7%), and the most commonly involved sites were the extremities (59.9%) and the scalp (46.8%). Limitations The population studied was a mostly white population in the upper Midwest. Conclusion The incidence of pediatric psoriasis increases with increasing age. There is no apparent dual peak in incidence. The incidence of pediatric psoriasis increased in recent years in both boys and girls.
Background Incidence studies of psoriasis are rare, mainly due to lack of established epidemiological criteria and the variable disease course. The objective of this study is to determine time trends ...in incidence and survival of psoriasis patients over three decades. Methods We identified a population-based incidence cohort of 1633 subjects aged ≥18 years first diagnosed with psoriasis between January 1, 1970 and January 1, 2000. The complete medical records for each potential psoriasis subject were reviewed and diagnosis was validated by either a confirmatory diagnosis in the medical record by a dermatologist or medical record review by a dermatologist. Age- and sex-specific incidence rates were calculated and were age- and sex-adjusted to the 2000 US white population. Results The overall age- and sex-adjusted annual incidence of psoriasis was 78.9 per 100,000 (95% confidence interval CI: 75.0-82.9). When psoriasis diagnosis was restricted to dermatologist-confirmed subjects, the incidence was 62.3 per 100,000 (95% CI: 58.8-65.8). Incidence of psoriasis increased significantly over time from 50.8 in the period 1970-1974 to reach 100.5 per 100,000 in the 1995-1999 time period ( P = .001). Although the overall incidence was higher in males than in females ( P = .003), incidence in females was highest in the sixth decade of life (90.7 per 100,000). Survival was similar to that found in the general population ( P = .36). Limitations The study population was mostly white and limited to adult psoriasis patients. Conclusion The annual incidence of psoriasis almost doubled between the 1970s and 2000. The reasons for this increase in incidence are currently unknown, but could include a variety of factors, including a true change in incidence or changes in the diagnosing patterns over time.
Background There are limited large case series of peristomal pyoderma gangrenosum (PPG), an uncommon cause of recalcitrant peristomal ulceration. Objective We sought to further characterize the ...clinical features, causes, treatments, and outcomes of PPG. Methods We conducted a retrospective chart review of patients with PPG seen at Mayo Clinic from January 1996 to July 2013. Results A total of 44 patients had PPG (mean age, 46 years; 32 women 73%); 41 (93%) had inflammatory bowel disease. Mean time to PPG onset after stoma surgery was 5.2 months (excluding 1 outlier). Systemic therapies included corticosteroids (66%), immunosuppressants (41%), biologics (36%), and a combination of systemic treatments (36%). Mean time to reach a complete response was 10.7 weeks. Stoma closure had the greatest complete response (4 of 4 patients, no recurrences). Recurrence after any treatment was documented in 23 of 38 (61%) patients. Stoma relocation/revision recurred in 10 of 15 (67%) patients. Remission occurred in 29 of 31 (94%) patients. Limitations Small sample size and retrospective study design are limitations. Conclusion PPG is strongly associated with inflammatory bowel disease, is predominant in women, and has a prolonged time to onset and high recurrence rate. Systemic corticosteroid or combination therapies and surgical closure can be effective treatments. Timely recognition and management are paramount to achieving early remission.
Background Postoperative pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by the development of PG-type lesions within surgical sites. Objective We sought to characterize ...postoperative PG as a distinct subtype of PG for earlier recognition and prevention of improper therapy. Methods We conducted a retrospective chart review of patients with nonperistomal postoperative PG at Mayo Clinic from 1994 to 2014.x Results Eighteen patients had postoperative PG with an average age of 58 years. Fifteen (83%) were female. Among patients with postoperative PG, 4 (22%) had an associated systemic disease traditionally associated with PG. Sites of postoperative PG included 7 breast (38%), 7 abdomen (38%), 1 back, 1 shoulder, 1 ankle, and 1 scrotum, witxxh breast reconstruction being the most common surgery. The average time to symptoms was 11 days. No patients had a fever. Eight (44%) had documented anemia and 5 (27%) had leukocytosis. Antibiotics and systemic corticosteroids were initiated in 10 (56%) and 14 (83%), respectively. Debridement was done in 11 (61%) patients. Limitations Small sample size and retrospective study are limitations. Conclusion Postoperative PG is a rare surgical complication with predilection for the breast and abdomen of females and has less association with systemic disease than idiopathic PG. Early recognition may prevent unnecessary debridements and morbidity.
Background Multiple devices and coatings assist with endovascular insertion of sheaths, catheters, and guide wires. Hydrophilic polymer coatings, a common component of endovascular surgical devices, ...reportedly cause microvascular obstruction and embolization, with various sequelae in organs and soft tissue. Objective We sought to describe clinical and histopathologic features of cutaneous manifestations of hydrophilic polymer gel emboli. Methods We evaluated the clinical and histopathologic characteristics of 8 patients with cutaneous complications of hydrophilic polymer gel emboli who presented in May 2013 through February 2015. Results Sudden onset of lower extremity livedo racemosa, purpuric patches, or both, occurred hours to days after endovascular procedures involving the aorta. Histopathologic evaluation showed basophilic lamellated material, consistent with hydrophilic polymer gel emboli, within small dermal vessels. Limitations This was a retrospective study with small sample size and not controlled for all similar procedures in this population. Conclusion Hydrophilic polymer gel coatings in endovascular devices can embolize to skin and cause microvascular occlusion, presenting as livedo racemosa, purpura, or both. Given the number of patients observed over a short period, this phenomenon may be underappreciated. Hydrophilic polymer gel emboli should be considered in differential diagnosis of livedo racemosa and purpura after endovascular procedure.
Background At our institution, hospitalization for intensive treatment (combining wet dressings and topical corticosteroids) is a primary intervention for severe pediatric atopic dermatitis. Prior ...reports of this treatment are limited. Objective We sought to review the efficacy of wet dressings for pediatric atopic dermatitis. Methods We reviewed records of pediatric patients hospitalized from January 1, 1980, through April 20, 2010, who received intensive topical treatments for atopic dermatitis. Results In total, 218 pediatric patients had widespread atopic dermatitis severe enough to warrant hospitalization, despite prior outpatient topical treatments and other interventions such as immunomodulating agents, phototherapy, dietary manipulation, or contact allergen avoidance. Mean (SD) age was 5.97 (4.91) years (range, 2 months-17 years); 141 patients (65%) were female. There were 266 hospitalizations: 192 patients (72%) had one admission, 15 (6%) had two admissions, and 11 (4%) had 3 or more admissions. Mean (SD) duration of hospitalization was 3.61 (2.23) days (range, 1-16 days). Upon discharge, all patients showed improvement. In 239 of 266 hospitalizations, patient records showed quantification of improvement (global assessment): 121 (45%) had 75% to 100% improvement, 102 (38%) had 50% to 75% improvement, and 16 (6%) had 25% to 50% improvement. Limitations This was a retrospective study. Conclusion Intensive inpatient treatment (with wet dressings and topical corticosteroids) was highly effective in controlling severe and recalcitrant atopic dermatitis. Intensive topical treatment, although underused, is an effective first-line approach for patients with severe atopic dermatitis.
Background Diffuse dermal angiomatosis (DDA) is a rare skin condition considered to be a type of reactive angioendotheliomatosis. Histologic features are quite characteristic. It has been reported in ...association with vaso-occlusive disease, trauma, or underlying hypercoagulability. In the past, it was thought to be most common on the lower extremities. Objective The purpose of this study was to describe the clinical and histologic features of 5 patients with DDA. Methods The clinical and histologic features of 5 patients with DDA were evaluated. Results Five women (47-58 years old) had DDA of the breast. Histologic examination showed a diffuse proliferation of benign endothelial cells between the collagen bundles throughout the dermis. Limitations The main limitation of our study is the limited number of patients. Conclusion Involvement of the breast is much more common than previously reported. Smoking seems to be a strong risk factor for the disease. Revascularization, oral corticosteroids, and oral anticoagulation have all been reported to be somewhat successful in the treatment of DDA of the breast.
Heart disease in psoriasis Kremers, Hilal Maradit, MD, MSc; McEvoy, Marian T., MD; Dann, Frank J., MD ...
Journal of the American Academy of Dermatology,
08/2007, Letnik:
57, Številka:
2
Journal Article
Recenzirano
Psoriasis has been traditionally viewed as an inflammatory skin disorder of unknown origin. Recent advances in the immunopathogenesis and genetics of psoriasis have broadened our understanding of ...psoriasis. Psoriasis is now considered a systemic inflammatory condition analogous to other inflammatory immune disorders. Patients with other immune disorders, such as systemic lupus erythematosus or rheumatoid arthritis, are known to be at increased risk of heart disease. Similarly, patients with psoriasis may carry an excess risk of heart disease, which would represent an important previously unrecognized cause of morbidity and mortality. This review summarizes the current evidence for an increased cardiovascular risk in patients with psoriasis and outlines deficits in our knowledge in this area.
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