Chronic recurrent wheals – If not chronic spontaneous urticaria, what else?
Hanna Bonnekoh1,2, Karoline Krause1,2, Pavel Kolkhir1,2
1 Institute of Allergology, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, and 2 Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
DOI 10.5414/ALX02375E
Abstract
Chronic urticarial rash, mostly due to chronic spontaneous urticaria (CSU), is seen in up to 1 – 4% of the general population. Urticarial vasculitis (UV) and autoinflammatory syndromes, i.e., cryopyrinassociated periodic syndromes (CAPS) and Schnitzler syndrome (SchS), can mimic CSUlike rash but represent rare disorders with systemic symptoms including fever, headache, conjunctivitis, and arthralgia. Clinical and laboratory features can point to the presence of any of these diseases in patients initially presenting with chronic urticarial rash. These include long-lasting wheals (> 24 hours), lesional burning, systemic symptoms, and/or increase in inflammatory markers (e.g., C-reactive protein, serum amyloid A, and/or S100A8/9). Lesional skin biopsy usually demonstrates leukocytoclastic vasculitis (UV) or neutrophil-rich infiltrate (CAPS and SchS). In contrast to CSU, where second-generation H1 antihistamines and omalizumab allow to control symptoms in most patients, systemic immunosuppression and anti-interleukin (IL)-1 therapies are needed in case of UV and autoinflammatory diseases, respectively. The rarity and low awareness of CSU differential diagnoses may be related to the longer delays in diagnosis and therapy in those affected with UV, CAPS, and SchS. Knowledge of the differential diagnoses of CSU is important because only correct diagnosis allows adequate therapy. Complications such as the development of lymphoproliferative disease in SchS and amyloidosis in CAPS, and the presence of comorbid diseases, such as systemic lupus erythematosus in UV, must be considered and monitored.
Author Details
Authors
Departments
- 1 Institute of Allergology, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, and
- 2 Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany
Address
Dr. med. Hanna Bonnekoh, Charite – Universitätsmedizin Berlin, Institut für Allergieforschung, Hindenburgdamm 30, 12203 Berlin, Germany
Email:
[email protected]
Citation
Hanna Bonnekoh, Karoline Krause, and Pavel Kolkhir.Chronic recurrent wheals – If not chronic spontaneous urticaria, what else?. Allergologie select. 2023; 7: 8-16. doi: 10.5414/ALX02375E.
