Behcet disease (BD) and BD-like clinical phenotypes : NF-kappa B pathway in mucosal ulcerating diseases

Behcet's disease (BD) is a heterogeneous multi-organ disorder in search of a unified pathophysiological theory and classification. The disease frequently has overlapping features resembling other disease clusters, such as vasculitides, spondyloarthritides and thrombophilias with similar genetic risk variants, namelyHLA-B*51,ERAP1,IL-10,IL-23R. Many of the BD manifestations, such as unprovoked recurrent episodes of inflammation and increased expression of IL-1, IL-6 and TNF alpha, overlap with those of the hereditary monogenic autoinflammatory syndromes, positioning BD at the crossroads between autoimmune and autoinflammatory syndromes. BD-like disease associates with various inborn errors of immunity, including familial Mediterranean fever, conditions related to dysregulated NF-kappa B activation (egTNFAIP3,NFKB1,OTULIN,RELA,IKBKG) and either constitutional trisomy 8 or acquired trisomy 8 in myelodysplastic syndromes. We review here the recent advances in the immunopathology of BD, BD-like diseases and the NF-kappa B pathway suggesting new elements in the elusive BD etiopathogenesis.Peer reviewe

Assessment of risk and use of prophylaxis for glucocorticoidinduced-osteoporosis among dermatologists in the Pacific Northwest: a survey study

Objective: Exposure to even physiologic doses of glucocorticoids can reduce one's bone mass and increase risk for osteoporotic fracture. There currently exists a wide variation in clinician approach to the assessment and management of glucocorticoid-induced osteoporosis (GIO). Our objectives were to characterize Pacific Northwest dermatology providers' general practices, assessment of risk for GIO, and preferred GIO prophylaxis measures by way of survey. To identify whether knowledge deficits exist with respect to preventing and managing GIO in dermatology patients.Design: A self-administered, 22-question survey was sent electronically to respondent population. Surveyed population composed of 392 dermatology providers of the Washington State Dermatology Association and Oregon Dermatology Society registries. Survey responses were collected anonymously via Catalyst WebQ.Results: Respondents over-estimated fracture risk and reported they would prescribe antiresorptive medications at a less-than-adequate rate. When given clinical scenarios and asked to assess risk of major osteoporotic fracture, respondents frequently overestimated risk compared to that estimated by the FRAX tool (67%-71%). When asked directly if one would prescribe bisphosphonates as GIO prophylaxis for a high-risk patient, only 49% responded always/almost always.Conclusions: This study suggests that a knowledge deficit exists within dermatology with respect to prevention and screening of GIO. The resultant practice gap is likely contributing to morbidity and mortality for dermatology patients requiring chronic glucocorticoid use for dermatologic disorders. Provider variability in practices suggests that dermatology could benefit from additional education in assessment and treatment of GIO, as well as a clear set of guidelines for GIO management

Assessment of risk and use of prophylaxis for glucocorticoidinduced-osteoporosis among dermatologists in the Pacific Northwest: a survey study

Objective: Exposure to even physiologic doses of glucocorticoids can reduce one's bone mass and increase risk for osteoporotic fracture. There currently exists a wide variation in clinician approach to the assessment and management of glucocorticoid-induced osteoporosis (GIO). Our objectives were to characterize Pacific Northwest dermatology providers' general practices, assessment of risk for GIO, and preferred GIO prophylaxis measures by way of survey. To identify whether knowledge deficits exist with respect to preventing and managing GIO in dermatology patients.Design: A self-administered, 22-question survey was sent electronically to respondent population. Surveyed population composed of 392 dermatology providers of the Washington State Dermatology Association and Oregon Dermatology Society registries. Survey responses were collected anonymously via Catalyst WebQ.Results: Respondents over-estimated fracture risk and reported they would prescribe antiresorptive medications at a less-than-adequate rate. When given clinical scenarios and asked to assess risk of major osteoporotic fracture, respondents frequently overestimated risk compared to that estimated by the FRAX tool (67%-71%). When asked directly if one would prescribe bisphosphonates as GIO prophylaxis for a high-risk patient, only 49% responded always/almost always.Conclusions: This study suggests that a knowledge deficit exists within dermatology with respect to prevention and screening of GIO. The resultant practice gap is likely contributing to morbidity and mortality for dermatology patients requiring chronic glucocorticoid use for dermatologic disorders. Provider variability in practices suggests that dermatology could benefit from additional education in assessment and treatment of GIO, as well as a clear set of guidelines for GIO management

Racial limitations of fitzpatrick skin type

Fitzpatrick skin type (FST) was developed to assess the propensity of the skin to burn during phototherapy, but it also is commonly used by providers as means of describing constitutive skin color and ethnicity. We conducted an anonymous survey of dermatologists and dermatology trainees to evaluate how providers use FST in their clinical practice. Although providers should be cognizant of conflating race/ethnicity with FST, the original intent of FST also should be emphasized in medical school and resident education

Cutaneous manifestations of angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin lymphoma often accompanied by autoimmune and paraneoplastic phenomena. Up to 50% of patients with AITL present with skin manifestations. This case series highlights two cases of AITL presenting with unusual cutaneous findings: one with a medium-vessel vasculitis and another with a chronic urticarial eruption. Clinicians should consider AITL in the differential diagnosis of vasculitis or urticaria refractory to standard treatment

Novel Diagnostic Educational Resource: Use of a web-based adaptive learning module to teach inflammatory reaction patterns in dermatopathology to medical students, residents, and fellows

Background: Perceptual and adaptive learning modules (PALM’s) provide a large number of visual examples for evaluation and accommodate to learner performance by actively adjusting the module parameters. Methods: We developed a module for discriminating 5 inflammatory reaction patterns using the Novel Diagnostic Educational Resource (NDER) platform. The module included a 20 question pre-test, a 200 question training section, and a 20 question post-test. During the pre-test and post-test, images were displayed for an indefinite period of time with no feedback given. In the training section, images were displayed for a duration inverse to learner performance, and after submitting their response learners were immediately shown the correct answer. The performance of module participants was compared to a control group who completed pre-test and post-test only. Results: 26 pathology and dermatology residents completed the module and were included in analysis. Pre-test and post-test scores showed an average increase of 17.1 percentage points (95% CI 13.0 to 21.2, P < 0.001). When performance on pre-test and post-test was compared between the module and control groups, module group performance increased more than control group performance by an average of 10.1 percentage points (95% CI -2.5 to 17.8, P = 0.0119). 84% (37) of participants found the module somewhat useful or very useful and 68% (30) of participants would be pretty likely or very likely to recommend to another trainee. Conclusions: Our findings validate the use of NDER for teaching inflammatory reaction patterns. Participants generally had favorable feedback regarding the interface and teaching potential of the module. Including a late re-test as part of the module would be beneficial in further validating future iterations. Next steps include optimizing module performance and developing module content for more advanced learners