Background
Multiple pilomatricomas have been linked to various syndromes. However, these associations are poorly defined, leaving practitioners conflicted on management of these patients.
Objective
To perform a comprehensive review to clarify the strength of these relationships and identify which patients may benefit from additional screening and/or genetic screening.
Methods
A literature search was performed using the PubMed, Ovid, and Cochrane databases. Syndromic, familial, and sporadic cases of multiple pilomatricomas were stratified based on number of pilomatricomas. This information was graphed for visual comparison.
Results
Sixty‐six syndromic cases from 52 publications were identified, with the majority (54) of cases representing myotonic dystrophy, familial adenomatous polyposis‐related syndromes (including Gardner syndrome), Turner syndrome, or Rubinstein‐Taybi syndrome. Twenty‐five of the 54 cases (46.3%) had six or more pilomatricomas. Of sporadic cases, 128 out of 134 (95.5%) had five or less pilomatricomas.
Limitations
Most articles were case reports and series, which are vulnerable to publication bias. Specific details were not explicitly noted in some original articles, and incomplete data could not always be included in analysis. Syndromes may have been missed in sporadic cases.
Conclusion
The presence of six or more pilomatricomas is highly suggestive of an underlying syndrome (>95% specificity). These patients should undergo additional screening. Patients with less than six pilomatricomas and family history of myotonic dystrophy, first‐degree relative with colon cancer or FAP‐related syndrome, or family history of pilomatricomas should also undergo further screening.
Pyoderma gangrenosum (PG) is a rare ulcerative skin disease of unknown etiology often associated with systemic inflammatory conditions, most commonly inflammatory bowel disease (IBD). The most common presentation of PG is spontaneous rapid formation of an extremely painful ulcer on the extremities, associated with significant morbidity and mortality. Rarely, PG can also occur as a chronic peristomal complication or as an acute postoperative complication of a surgical wound. The clinical course is unpredictable; it may not correlate with IBD activity and may even precede a diagnosis of IBD. Pyoderma gangrenosum is a diagnosis of exclusion. Treatment is challenging, often involving a variety of immunosuppressive therapies. This review aims to provide an update for the gastroenterologist on the pathogenesis, presentation, diagnosis, and management of PG, a rare complication of IBD.
IMPORTANCEFirst-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. When this regimen is ineffective, not tolerated, or contraindicated, a trial of mycophenolate mofetil (MMF) or mycophenolic acid (MPA)-referred to herein as mycophenolate-is recommended; however, evidence to support this recommendation remains weak.OBJECTIVE To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea.
Mycobacterium abscessus and M. chelonae belong to the rapid‐growing nontuberculous mycobacteria (NTM) group, which are defined by their ability to form visible colonies on agar within 7 days of subculture. Cutaneous infections by this complex show a heterogeneous clinical presentation with varied histopathologic findings. However, the presence of vacuoles in many specimens has been reported as a specific histologic finding. Herein, we correlate the histopathology of patients with tissue‐culture positive M. abscessus/M. chelonae complex in order to identify features that may prompt a rapid categorization of the infectious etiology. The cohort includes 33 skin punch biopsy specimens from 28 patients who had associated positive tissue cultures. The most frequent clinical presentation was a single or multiple nodule. Twenty‐seven specimens (81.81%) were found to have vacuoles. The observation of certain histologic features (ie, polymorphonuclear microabscesses and epithelioid granuloma formation) should raise the possibility of infection by NTM. In addition to these findings, we believe the presence of vacuoles in the dermal and subcutaneous inflammation should raise suspicion for NTM.
BackgroundSedentary aging leads to adverse changes in vascular function and cardiac performance. We published improvements in vascular function with moderate intensity physical activity (PA) in continuous bouts. Whether moderate intensity PA also impacts cardiac structure and cardiovascular performance of the aging left ventricle (LV) is unknown.MethodsWe recruited and analyzed results from 102 sedentary older adults ages ≥ 50 from a randomized controlled trial with 3 study groups: control (group 1), a pedometer-only intervention (group 2), or a pedometer with an interactive website employing strategies to increase habitual physical activity (PA, group 3) for 12 weeks. Transthoracic echocardiograms were performed prior to and following the 12 week intervention period to assess cardiac morphology, left ventricular (LV) systolic performance, LV diastolic function, and arterial and LV ventricular elastance. Step count and PA intensity/distribution were measured by a pedometer and an accelerometer.ResultsWe found no significant changes in cardiac morphology. Further, we found no improvement in the aforementioned cardiac functional parameters. Comparing those who achieved the following benchmarks to those who did not showed no significant changes in cardiac structure or performance: 1) 10,000 steps/day, 2) ≥ 30 min/day of moderate intensity physical activity, or 3) moderate intensity PA in bouts ≥ 10 min for ≥ 20 min/dayConclusionsIn sedentary older adults, increasing moderate intensity PA to currently recommended levels does not result in favorable changes in LV morphology or performance over 12 weeks. More prolonged exposure, higher PA intensity, or earlier initiation of PA may be necessary to see benefits.
Reductions of up to 100 minutes of sedentary time per day over 12 weeks was not significantly associated with improved vascular endothelial function in older adults. FMD% was significantly higher among those with lower sedentary behavior and concomitant moderate-intensity PA of ≥20 minutes/day in bouts.
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