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Assessment of Cutaneous and Mucosal Direct Immunofluorescence Testing Practices in the US

Lehman, Julia S; Fernandez, Anthony P; Leiferman, Kristin M; Brinster, Nooshin K; Culton, Donna A; Kim, Randie H; North, Jeffrey P; Stoff, Benjamin K; Camilleri, Michael J; Cocks, Margaret M; Elenitsas, Rosalie; Fung, Maxwell A; Grover, Raminder K; Jedrych, Jaroslaw J; Kuechle, Melanie K; McNiff, Jennifer M; Moshiri, Ata S; Motaparthi, Kiran; Murphy, Michael J; Nousari, Carlos H; Shalin, Sara C; Zone, John J; Bridges, Alina G
IMPORTANCE/UNASSIGNED:Direct immunofluorescence (DIF) testing has been an important ancillary tool for the diagnosis of various inflammatory mucocutaneous conditions for more than 50 years. Current DIF test panels are based on historical clinical descriptions; few studies have rigorously addressed preanalytical, analytical, and/or postanalytical aspects, and even fewer have been replicated or validated. Recent unresolved key issues include whether DIF testing and test panels should be triaged or truncated based on clinical indication or histopathologic findings. OBJECTIVE/UNASSIGNED:To assess levels of consensus regarding practical aspects of DIF testing among immunodermatology testing specialists in the US. DESIGN, SETTING, AND PARTICIPANTS/UNASSIGNED:Using modified Delphi methods with a priori characterized criteria, a survey containing 54 statements pertaining to DIF testing was created and distributed to assess consensus. Statements not initially reaching consensus were discussed in 2 live virtual sessions, which were supplemented by relevant literature review and free-text survey comments. These statements were then reassessed in a second survey. Immunodermatology testing specialists in US academic institution-based and independent laboratories were invited based on serving as immunodermatology laboratory medical directors, authoring pertinent literature, or delivering relevant talks at major conferences or by referral. The first survey was conducted from January to February 2024, and the second survey was conducted from March to April 2024. MAIN OUTCOMES AND MEASURES/UNASSIGNED:The primary measured outcome was degree of consensus for various DIF testing practice, including DIF testing triage by histopathology/dermatopathology findings and DIF testing panel tailored truncations by clinical indication. RESULTS/UNASSIGNED:A total of 23 respondents to the survey invitation had a mean (SD) of 18.5 (11.1) years and median (range) of 20.0 (1.5-46.0) years in immunodermatology laboratory practice. Consensus was achieved for 46 of 54 statements (85.2%) in the initial survey and for an additional 4 statements in the second survey (50 of 54 [92.6%]). Strong consensus was found against tailored truncation of DIF panel based on the clinical indication in the first survey round. The general acceptability of triaging specimens for DIF testing based on histopathology findings remained without consensus after both surveys. CONCLUSIONS AND RELEVANCE/UNASSIGNED:Overall, participating US specialists in immunodermatology laboratory testing agreed on many practical aspects of DIF testing, including matters not queried previously. The findings also revealed areas of continued controversy and identified issues for prioritized future study.
PMID: 40172897
ISSN: 2168-6084
CID: 5819122

Differentiating and Managing Cutaneous Lupus Erythematosus-Associated Alopecia and Patchy Alopecia Areata: Therapeutic Insights From Case Studies [Case Report]

G Buontempo, Michael; Alhanshali, Lina; Milam, Emily; Caplan, Avrom; Shapiro, Jerry; Alvarez, Anna; Kim, Randie; Lo Sicco, Kristen
Cutaneous lupus erythematosus (CLE) is an autoimmune disease with diverse clinical manifestations, including patchy hair loss resembling alopecia areata (AA). This report describes two cases of CLE presenting as AA mimickers, emphasizing the need to consider CLE in differential diagnosis for patchy hair loss. Early and accurate diagnosis is crucial for effective management and preventing scarring alopecia. J Drugs Dermatol. 2025;24(3):324-326. doi:10.36849/JDD.7793R1.
PMID: 40043260
ISSN: 1545-9616
CID: 5843212

Linear IgA bullous dermatosis induced by nemvaleukin alfa, an engineered interleukin 2 molecule, in a patient with treatment-refractory metastatic melanoma [Case Report]

Bawany, Fatima; Ramachandran, Vignesh; Rodriguez, Eduardo; Kim, Randie H; Weber, Jeffrey S; Tattersall, Ian W
PMCID:10568223
PMID: 37842150
ISSN: 2352-5126
CID: 5684742

Diagnosing mycosis fungoides after initiation of therapy with dupilumab: a case report and literature review [Letter]

Yan, Di; Ramachandran, Vignesh; Weston, Gillian; Kim, Randie H; Latkowski, Jo-Ann
PMID: 36946434
ISSN: 1365-4632
CID: 5684682

A case of cutis verticis gyrata developing in a patient with primary scarring alopecia: A unique presentation of a rare disorder [Case Report]

Buontempo, Michael G; Alhanshali, Lina; Shapiro, Jerry; Klein, Elizabeth J; Oh, Christina S; Kim, Randie H; Rodriguez, Eduardo A; Lo Sicco, Kristen
PMCID:10338184
PMID: 37448472
ISSN: 2352-5126
CID: 5537842

Pruritic Rash in a Woman With Endometrial Cancer

Liang, Sydney E; Kim, Randie H
PMID: 36753090
ISSN: 2168-6084
CID: 5447982

Eosinophil-rich linear IgA bullous dermatosis induced by mRNA COVID-19 booster vaccine [Case Report]

Nahm, William J; Juarez, Michelle; Wu, Julie; Kim, Randie H
We present a case of eosinophil-rich linear IgA bullous disease (LABD) following administration of an mRNA COVID-19 booster vaccine. A 66-year-old man presented to the Emergency Department with a 3-week history of a pruritic blistering rash characterized by fluid-filled bullae and multiple annular and polycyclic plaques. He was initially diagnosed with bullous pemphigoid based on a biopsy demonstrating a subepidermal blister with numerous eosinophils. However, direct immunofluorescence studies showed linear IgA and IgM deposition along the basement membrane zone with no immunoreactivity for C3 or IgG. Additionally, indirect immunofluorescence was positive for IgA basement membrane zone antibody. The patient was subsequently diagnosed with LABD and initiated on dapsone therapy with resolution of his lesions at three-month follow-up. This case illustrates the growing number of autoimmune blistering adverse cutaneous reactions from vaccination. Dermatopathologists should be aware that features of autoimmune blistering diseases can overlap and may not be distinguishable based on these histopathological findings alone. Confirmation with direct immunofluorescence and/or serological studies may be necessary for accurate diagnosis. This article is protected by copyright. All rights reserved.
PMID: 35922892
ISSN: 1600-0560
CID: 5288132

Diagnostic work-up and treatment in patients with pyoderma gangrenosum: retrospective analysis of US insurance claims-based data

Shaigany, Sheila; Wong, Priscilla W; Caplan, Avrom; Kim, Randie H; Femia, Alisa
Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and "real-world" practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.
PMID: 34714405
ISSN: 1432-069x
CID: 5042872

Mohs micrographic surgery reduces the need for a repeat surgery for primary Merkel cell carcinoma when compared to wide local excision: A retrospective cohort study of a commercial insurance claims database

Tran, Duy C; Ovits, Channa; Wong, Priscilla; Kim, Randie H
PMCID:9558042
PMID: 36248207
ISSN: 2666-3287
CID: 5360152

Scaly Plaques on the Foot: Challenge

Roman, Jorge; Kim, Randie H; Marji, Jackleen S
PMID: 36122344
ISSN: 1533-0311
CID: 5333062