Reactive Neutrophilic Dermatoses in Adult-Onset Immunodeficiency due to Interferon-Gamma Autoantibody and Their Associated Factors

Tungphaisal V.a· Phinyo P.b,c· Rujiwetpongstorn R.d· Kiratikanon S.d· Tovanabutra N.d· Chaiwarith R.e· Chiewchanvit S.d· Chuamanochan M.d,f

Author affiliations

aDepartment of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
bCenter for Clinical Epidemiology and Clinical Statistics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
cDepartment of Family Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
dDivision of Dermatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
eDivision of Infectious Diseases and Tropical Medicine, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
fPharmacoepidemiology and Statistics Research Center (PESRC), Faculty of Pharmacy, Chiang, Mai University, Chiang Mai, Thailand

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Article / Publication Details

First-Page Preview

Abstract of Research Article

Received: May 02, 2022
Accepted: November 11, 2022
Published online: January 18, 2023

Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 5

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: https://www.karger.com/DRM

Abstract

Background: Adult-onset immunodeficiency (AOID) due to interferon-gamma autoantibody is a rare, acquired immunodeficiency disease. Reactive neutrophilic dermatoses (RND), predominantly Sweet syndrome (SS), and generalized pustular eruption have been reported repeatedly. Objectives: The aims of this study were to describe the cutaneous manifestations in AOID patients and determine the incidence of RND and associated factors using a larger population size than have been previously reported. Methods: A retrospective chart review of all confirmed AOID cases in Chiang Mai University Hospital from January 2006 to June 2020 was conducted. The demographics and characteristics of RND including type, onset, and laboratory information in every episode of cutaneous manifestations were collected. Generalized estimating equations of binary logistic regression were used to determine the indicators of RND. Results: A total of 146 patients with confirmed AOID were identified. Of these, 57 cases (39%) developed at least one episode of RND. Thirteen cases (23%) of the patients experienced RND twice during the follow-up period. All recurrence of RND displayed the same cutaneous phenotype, with the exception of 2 cases who had both SS and generalized pustular eruption. Finally, 49 episodes of SS and 22 episodes of generalized pustular eruption were included in the analysis. All patients with RND had concomitant active opportunistic infections, of which most were non-tuberculous mycobacterium (NTM) infection. NTM infection (prevalence odds ratio [POR] 2.87), lymphadenopathy (POR 3.30) as well as lower serum alkaline phosphatase (ALP) level (POR 0.71 for every 100-unit increment in ALP) were found to be significantly associated with RND occurrence. Conclusions: 39% of our AOID patients experienced RND once during the course of the disease. Notable factors associated with RND occurrence were concomitant NTM infection, lymphadenopathy, and lower level of ALP.

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First-Page Preview

Abstract of Research Article

Received: May 02, 2022
Accepted: November 11, 2022
Published online: January 18, 2023

Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 5

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: https://www.karger.com/DRM

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