Faculty Bibliography

BACKGROUND:Atopic diseases are characterized by IgE antibody responses that are dependent on cognate CD4 T cell help and T cell-produced IL-4 and IL-13. Current models of IgE cell differentiation point to the role of IgG memory B cells as precursors of pathogenic IgE plasma cells. The goal of this work was to identify intrinsic features of memory B cells that are associated with IgE production in atopic diseases. METHODS:Peripheral blood B lymphocytes were collected from individuals with physician diagnosed asthma or atopic dermatitis (AD) and from non-atopic individuals. These samples were analyzed by spectral flow cytometry, single cell RNA sequencing (scRNAseq), and in vitro activation assays. RESULTS:cells than B cells from non-atopic subjects. CONCLUSIONS:memory B cells transcribing IGHE are potential precursors of IgE plasma cells and are linked to pathogenic IgE production.

Methotrexate is historically recognized as an effective treatment of pemphigus but its utility as a single or alternate steroid-sparing agent was not recognized in recent consensus recommendations in pemphigus management. We aimed to evaluate the efficacy and safety of a treatment course for pemphigus that involves methotrexate as a single or steroid-sparing agent. In a retrospective cohort study, we examined patients with pemphigus vulgaris or pemphigus foliaceus who were on ≥3 months of methotrexate therapy. Efficacy and safety were evaluated by established pemphigus disease endpoints. Of the 34 patients who met inclusion criteria, 25 (73.5%) were on glucocorticoids at time of methotrexate initiation (median follow-up: 5.4 years; median time on methotrexate: 3.7 years). An appreciable proportion achieved disease control (91.2%), with some achieving clinical remission off all systemic therapies (23.5%). For patients on glucocorticoids, median time to control was 42 days, median time to minimal steroid dose tapering (5 mg prednisone) was 161 days, and median time to complete steroid tapering was 308 days. For patients on methotrexate as a single agent, median time to control was 119 days. Among all patients, relapse commonly occurred (88.2%). At last follow-up, 26.5% were managed on topical therapies alone and 11.8% required systemic steroid therapy. Methotrexate was largely tolerated with a low incidence of adverse events leading to treatment discontinuation (2.9%). Methotrexate has the potential to be an effective and well-tolerated option for patients and may be considered for use as an alternate single or steroid-sparing agent for pemphigus.

BACKGROUND:The first-line treatment for patients with bullous pemphigoid (BP), the most common autoimmune blistering disease, is systemic glucocorticoids, which are associated with numerous side effects. Mycophenolate mofetil (MMF) may be beneficial in BP as a steroid-sparing alternative; however, evidence is limited. OBJECTIVES/OBJECTIVE:To evaluate the efficacy and safety of MMF in patients with BP. METHODS:In this retrospective chart review, records of patients with BP treated with MMF alone or in combination with prednisone, who presented between 2013 and 2017, were analyzed. RESULTS:Twenty-six patients were included. Twelve patients were treated with MMF alone (monotherapy) and fourteen were treated with MMF and prednisone concomitantly at some point during their treatment course (dual therapy). Improvement in BP was observed in 26 (100%) patients with MMF therapy. Mean time to improvement was 0.8 months. Twenty-five (96.2%) patients [11/12 (91.7%) on monotherapy and 14/14 (100%) on dual therapy] achieved complete control of their disease. Mean time to complete control amongst all patients was 5.6 months. Twelve (46.2%) patients [4/12 (33.3%) on monotherapy and 8/14 (57.1%) on dual therapy] experienced disease remission with no subsequent flares for up to 15 months after MMF was discontinued. Twelve mild adverse effects were reported with one individual discontinuing therapy due to gastrointestinal symptoms. No serious adverse effects were reported. CONCLUSION/CONCLUSIONS:MMF is a safe and effective therapy for BP and can yield improvement and complete response in most patients and remission in some. J Drugs Dermatol. 2022;21(2):151-155. doi:10.36849/JDD.6042.

Darier disease is an autosomal dominant genodermatosis of abnormal keratinization characterized by hyperkeratotic papules and plaques with a predilection for seborrheic areas. We report a case of a rare vesiculobullous variant of treatment-resistant Darier disease in a 55-year-old woman that failed topical tacrolimus and topical and oral glucocorticoids. Cetirizine was initiated at 10 mg daily and increased to 40 mg daily over four weeks, with resultant marked improvement of the patient’s burning sensation. A punch biopsy revealed a perivascular infiltrate of eosinophils. This patient’s symptomatic improvement with cetirizine, which has antagonizing properties against eosinophils, highlights the potential role of eosinophils in the pathogenesis of vesiculobullous Darier disease. We suggest that major basic protein secreted by eosinophils may propagate blister formation in vesiculobullous Darier disease by disrupting desmosomes.

Importance/UNASSIGNED:The first-line treatment for patients with chronic spontaneous urticaria (CSU), which is divided into idiopathic and autoimmune subtypes, consists of H1-antihistamines. However, limited evidence guides the treatment of CSU after maximal therapy with antihistamines fails. Two randomized clinical trials suggest that dapsone may be a successful second-line therapy. Objective/UNASSIGNED:To evaluate the efficacy and safety of dapsone therapy in patients with CSU. Design, Setting, and Participants/UNASSIGNED:This retrospective medical record review included 79 patients with CSU treated with dapsone who presented to the tertiary care academic medical center at the New York University School of Medicine, New York, New York, from January 1, 2005, through April 15, 2017. Follow-up was completed on February 28, 2018. Data were analyzed from March 1 through May 31, 2018. Exposures/UNASSIGNED:Treatment with oral dapsone for CSU. Main Outcomes and Measures/UNASSIGNED:Efficacy of dapsone therapy for CSU was evaluated as improvement, complete response, and remission. Results/UNASSIGNED:Seventy-nine patients (65% women; mean [SD] age, 49.8 [16.1] years [range, 20-79 years]) were included in the analysis. Forty-five patients had chronic idiopathic urticaria and 34 had chronic autoimmune urticaria. Improvement in CSU was observed in 62 patients (78%) (36 [80%] with idiopathic and 26 [76%] with autoimmune disease) with dapsone. Mean (SD) time to improvement was 1.1 (1.0) months. A complete response was achieved in 29 (47%) of these 62 patients (16 [44%] with idiopathic and 13 [50%] with autoimmune disease). Mean (SD) time to complete response was 5.2 (5.2) months. Dapsone therapy was tapered in 21 patients after a mean (SD) of 2.4 (2.2) months and discontinued in 18. Ten patients experienced remission with no subsequent flares, even after dapsone therapy was discontinued with follow-up of 0.3 to 10.0 months. Sixteen patients experienced mild adverse effects. Two serious adverse effects were reported. Conclusions and Relevance/UNASSIGNED:Results of this study suggest that dapsone is a useful and well-tolerated second-line therapy for patients with CSU in whom antihistamines and other first-line agents have failed.

We present a 38-year-old woman with local heat urticaria confirmed by heat provocation testing. Heat urticaria is a rare form of physical urticaria that istriggered by exposure to a heat source, such as hot water or sunlight. Although it is commonly localized and immediate, generalized and delayed onset forms exist. Treatment options include antihistamines and heat desensitization. A brisk, mechanical stroke elicited a linear wheal. Five minutes after exposure to hot water, she developed well-demarcated,erythematous blanching wheals that covered the distal forearm and entire hand.