TY - JOUR
T1 - Subtypes of Atypical Cold Urticaria and Recommendations for Their Diagnostic Workup
AU - Ahsan, Dalia M
AU - Elieh-Ali-Komi, Daniel
AU - Pereira, Manuel P
AU - Sürmeli, Seda
AU - Bizjak, Mojca
AU - Brockstaedt, Maxi
AU - Metz, Martin
AU - Altrichter, Sabine
AU - Terhorst-Molawi, Dorothea
N1 - Copyright © 2025. Published by Elsevier Inc.
PY - 2025/9
Y1 - 2025/9
N2 - BACKGROUND: A substantial subset of patients suffering from cold urticaria (ColdU) have atypical disease presentations and develop cold-induced wheals and/or angioedema in a real-world setting but not in response to standard cold stimulation tests. How to diagnose atypical ColdU is largely unclear, and little is known about the clinical features and treatment responses.OBJECTIVE: The aim of the study was to describe subtypes of atypical ColdU and provide recommendations for the diagnostic workup.METHODS: We conducted a comprehensive literature search on subtypes of atypical ColdU and published testing procedures. We then developed and pilot-tested modified cold stimulation tests (mCSTs) for 8 subtypes of atypical ColdU.RESULTS: Our systematic review identified 10 subtypes of atypical ColdU-delayed ColdU, ColdU with aberrant wheals, extended exposure ColdU, generalized exposure ColdU, localized ColdU, cold-induced cholinergic urticaria, cold-dependent dermographism, water-dependent ColdU, food-dependent ColdU, and ultra-freeze ColdU. Pilot-testing of protocols for diagnostic mCSTs was successful in 8 of 8 variants. Based on these results, we generated recommendations for the diagnostic workup of known subtypes of atypical ColdU.CONCLUSIONS: The mCSTs provide a more precise diagnostic confirmation in patients with suspected atypical ColdU and allow effective monitoring throughout the disease course, facilitating access to specific therapy options, and ultimately leading to improved care for this patient population.
AB - BACKGROUND: A substantial subset of patients suffering from cold urticaria (ColdU) have atypical disease presentations and develop cold-induced wheals and/or angioedema in a real-world setting but not in response to standard cold stimulation tests. How to diagnose atypical ColdU is largely unclear, and little is known about the clinical features and treatment responses.OBJECTIVE: The aim of the study was to describe subtypes of atypical ColdU and provide recommendations for the diagnostic workup.METHODS: We conducted a comprehensive literature search on subtypes of atypical ColdU and published testing procedures. We then developed and pilot-tested modified cold stimulation tests (mCSTs) for 8 subtypes of atypical ColdU.RESULTS: Our systematic review identified 10 subtypes of atypical ColdU-delayed ColdU, ColdU with aberrant wheals, extended exposure ColdU, generalized exposure ColdU, localized ColdU, cold-induced cholinergic urticaria, cold-dependent dermographism, water-dependent ColdU, food-dependent ColdU, and ultra-freeze ColdU. Pilot-testing of protocols for diagnostic mCSTs was successful in 8 of 8 variants. Based on these results, we generated recommendations for the diagnostic workup of known subtypes of atypical ColdU.CONCLUSIONS: The mCSTs provide a more precise diagnostic confirmation in patients with suspected atypical ColdU and allow effective monitoring throughout the disease course, facilitating access to specific therapy options, and ultimately leading to improved care for this patient population.
KW - Humans
KW - Urticaria/diagnosis
KW - Cold Temperature/adverse effects
KW - Cold Urticaria
UR - https://www.scopus.com/pages/publications/105015454721
U2 - 10.1016/j.jaip.2025.07.019
DO - 10.1016/j.jaip.2025.07.019
M3 - Article
C2 - 40706706
SN - 2213-2201
VL - 13
SP - 2370-2380.e2
JO - The Journal of Allergy and Clinical Immunology: In Practice
JF - The Journal of Allergy and Clinical Immunology: In Practice
IS - 9
ER -