Faculty Bibliography

Cutaneous lupus erythematosus (CLE) is a chronic skin disease that significantly impacts quality of life (QOL). This study tested a novel method to measure QOL in CLE using willingness-to-pay (WTP) stated preferences, and aimed to determine which of nine domains of life are most affected by CLE. Twenty-one participants with CLE ranked the domains in order of impact on CLE-related QOL, and then stated how many United States dollars they would be willing to pay for a hypothetical cure for each domain. Eighty-one percent of participants were female; mean age was 42.4 years. Photosensitivity was ranked highest by 71.4% of respondents. Participants were willing to pay the most for a hypothetical cure for CLE specific to photosensitivity (median = $200,000), the least for a cure specific to self-care (median = $0). Participants were willing to pay a median of $1,000,000 for an overall cure for CLE. Limitations include a small sample size for this pilot study and that willingness-to-pay may be influenced by individual perception of money and socioeconomic factors. This study successfully pilot-tested a WTP method and ranking task for health-related QOL. Photosensitivity was the domain of life most affected by CLE, which is a domain unique to our novel tool.

Dermatomyositis is a rare inflammatory disease with characteristic cutaneous findings and varying amounts of systemic involvement. Patients may present with skin disease alone, have concomitant muscle disease, or have extracutaneous manifestations such as pulmonary disease or an associated malignancy. Given such diverse presentations, dermatomyositis is both a diagnostic and therapeutic challenge. However, a prompt diagnosis is of utmost importance to institute adequate therapy and screen patients for an associated malignancy. Dermatologists should play a crucial role in the diagnosis and management of patients with dermatomyositis as cutaneous disease tends to be chronic, negatively impact quality of life, and be more recalcitrant to therapy. In this review, we discuss diagnosis, with a focus on myositis-specific antibodies and their associated phenotypes. We also review therapies available for this often refractory skin disease.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists (SDH) with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. 9-point Likert scale questionnaires regarding 135 statements were administered. The RAND/UCLA appropriateness method was employed to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5-9 and disagreement index ≤1 were included in the guideline. For the final round, the guidelines were appraised by all the participants. An evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN are included.

BACKGROUND:In instances of suspected cutaneous infection, standard of care includes obtaining skin biopsies for histology and tissue culture. Few studies have compared the clinical utility of each test. OBJECTIVE:To assess the concordance of results between tissue culture and histology, and clinicopathologic features that may influence the diagnostic yield of each test. METHODS:A retrospective review of all patients who underwent skin biopsy for histology and tissue culture at New York University from 2013-2018. RESULTS:Of 179 patients, 10% had positive concordance, 21% had positive tissue culture only, and 7% had positive histology only. We calculated a kappa correlation coefficient of 0.25 between histology and tissue culture [ref 0.21-0.39=minimal agreement]. Histology exhibited higher sensitivity in detecting fungi, whereas tissue culture was more sensitive in identifying gram-negative bacteria. Antimicrobial use prior to biopsy led to significantly fewer positive cultures (37.5% versus 71%; p=0.023) in patients ultimately diagnosed with infection. LIMITATIONS/CONCLUSIONS:This study was conducted at a single institution thereby restricting its broad applicability. The lack of a validated gold standard to diagnose infection also limits interpretation of results. CONCLUSION/CONCLUSIONS:Tissue culture and histopathology often yield discordant results. Dermatologists should recognize specific test limitations, yet high clinical utility in special circumstances, when approaching cases of suspected infection.

Importance/UNASSIGNED:First-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/UNASSIGNED:To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea. Design, Setting, and Participants/UNASSIGNED:A retrospective cohort study was conducted from January 1, 1999, to December 31, 2018, among 77 patients with morphea from 8 institutions who were treated with mycophenolate. Main Outcomes and Measures/UNASSIGNED:The primary outcome was morphea disease activity, severity, and response at 0, 3 to 6, and 9 to 12 months of mycophenolate treatment. A secondary outcome was whether mycophenolate was a well-tolerated treatment of morphea. Results/UNASSIGNED:There were 61 female patients (79%) and 16 male patients (21%) in the study, with a median age at disease onset of 36 years (interquartile range, 16-53 years) and median diagnostic delay of 8 months (interquartile range, 4-14 months). Generalized morphea (37 [48%]), pansclerotic morphea (12 [16%]), and linear morphea of the trunk and/or extremities (9 [12%]) were the most common subtypes of morphea identified. Forty-one patients (53%) had an associated functional impairment, and 49 patients (64%) had severe disease. Twelve patients received initial treatment with mycophenolate as monotherapy or combination therapy and 65 patients received mycophenolate after prior treatment was ineffective (50 of 65 [77%]) or poorly tolerated (21 of 65 [32%]). Treatments prior to mycophenolate included methotrexate (48 of 65 [74%]), systemic corticosteroids (42 of 65 [65%]), hydroxychloroquine (20 of 65 [31%]), and/or phototherapy (14 of 65 [22%]). After 3 to 6 months of mycophenolate treatment, 66 of 73 patients had stable (n = 22) or improved (n = 44) disease. After 9 to 12 months of treatment, 47 of 54 patients had stable (n = 14) or improved (n = 33) disease. Twenty-seven patients (35%) achieved disease remission at completion of the study. Treatments received in conjunction with mycophenolate were frequent. Mycophenolate was well tolerated. Gastrointestinal adverse effects were the most common (24 [31%]); cytopenia (3 [4%]) and infection (2 [3%]) occurred less frequently. Conclusions and Relevance/UNASSIGNED:This study suggests that mycophenolate is a well-tolerated and beneficial treatment of recalcitrant, severe morphea.