Summary Background The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin‐predominant dermatomyositis (DM). However, approximately 25% of patients with skin‐predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria. Objectives To develop a set of skin‐focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin‐predominant disease. Methods An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank‐ordered list. A prespecified median score cut‐off was decided by the steering committee and the participants. There was a pre‐Delphi and two rounds of actual Delphi. Results There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut‐off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut‐off was raised to 80 during round two and a list of 25 items was generated. Conclusions This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin‐predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin‐predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin‐predominant DM.

Background: Long-term studies characterizing disease course of cutaneous lupus erythematosus (CLE) patients on standard-of-care treatments are lacking. Objective: We characterized and compared disease course of CLE patients using Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Methods: In total, 83 CLE patients with CLASI scores collected from ≥3 study visits within 2 years had disease activity and damage trends calculated by average change scores (ACS). Trends were classified as improved (ACS ≤−3), worsened (ACS ≥3), or stable (−3 < ACS < 3). Linear regression models compared CLASI trends between groups. Results: Most patients (72.73%) with initial CLASI activity (CLASI-A) scores >9 (N = 33) had improved disease activity versus 14.00% of those with initial CLASI-A scores ≤9 (N = 50). Linear regression analyses showed significant improvement in CLASI-A scores in patients of minority races (P < .05), with baseline CLASI-A scores >9 (P <.0001), baseline CLASI damage (CLASI-D) scores ≥10 (P = .0001), and CLE disease duration ≤1 year (P = .01). Of 28 patients with baseline CLASI-D scores ≥10, 35.71% had improvements in damage, while 5.26% of patients with initial CLASI-D scores of 5–9 (N = 19) and 0% with initial CLASI-D scores <5 (N = 36) (P = .0005) had improvements. Limitations: Limitations include small sample size. Conclusion: Baseline CLASI-A score >9, minority race, and short disease duration predict CLE disease activity improvement. A baseline CLASI-D score ≥10 is associated with disease damage improvement.

Adult-onset AP is more common in the Asian population, with a male predominance. The face, forearms, and hands are the most commonly affected areas. The absence of mucosal involvement is also a distinguishing feature between the Asian and Caucasian population. Close to half of the patients have reduced MED to UVA on phototesting. The prognosis for AP is poor as it tends to run a chronic course with suboptimal response to treatment.

No abstract