Cold-induced urticaria in a child with familial Mediterranean fever

Abstract

We read the article “Acquired cold-induced urticaria in pediatric patients: A 22-year experience in a tertiary care center (1996-2017)” with great interest.1Yee C.S.K. El Khoury K. Albuhairi S. Broyles A. Schneider L. Rachid R. Acquired cold-induced urticaria in pediatric patients: a 22-year experience in a tertiary care center (1996-2017).J Allergy Clin Immunol Pract. 2019; 7: 1024-1031Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar The authors reported the association of autoimmune diseases in 3.6% of their patients; however, there was no patient with cold-induced urticaria and autoinflammatory disease. We would like to share our experience with a patient who presented with typical attacks of familial Mediterranean fever (FMF) except for urticaria triggered by cold, occurring mostly during attacks. A 12-year-old boy admitted to our hospital with recurrent attacks of fever, abdominal pain, chest pain, and urticarial rash. These attacks were present since he was 3 years old. He was compound heterozygous for M694V and R761H mutations on the MEFV gene. He had been using colchicine since he was 4 years with the diagnosis of FMF. The attack frequency significantly decreased with colchicine. With the clinical findings and genetic test results, FMF diagnosis was certain; however, urticaria during attacks was atypical for FMF. His mother explained that he had rash only in the locations where she applied cold cloths to reduce fever during attacks. He also had rash in attack-free periods with direct contact to cold water/objects and exposure to cool air. With this information, we suspected cold-induced urticaria and performed ice-cube challenge test (ICCT), the result for which was positive at 10 minutes. He did not have cough, dyspnea, or hypotension triggered by cold. We diagnosed cold-induced urticaria as an associated condition in this patient with FMF. This is the first report of such co-occurrence. Avoidance of cold exposure and use of nonsedating H1-antihistaminic drugs (on demand) were recommended to the patient. FMF is an autosomal-recessive autoinflammatory disease characterized by recurrent attacks of fever and polyserositis caused by mutations in the MEFV gene.2Sonmez H.E. Batu E.D. Ozen S. Familial Mediterranean fever: current perspectives.J Inflamm Res. 2016; 9: 13-20PubMed Google Scholar, 3Ozen S. Batu E.D. Demir S. Familial Mediterranean fever: recent developments in pathogenesis and new recommendations for management.Front Immunol. 2017; 8: 253Crossref PubMed Scopus (93) Google Scholar Rash is very rare during FMF attacks. The only cutaneous manifestation of FMF is erysipelas-like erythema, which mostly occurs around the ankles.4Lidar M. Doron A. Barzilai A. Feld O. Zaks N. Livneh A. et al.Erysipelas-like erythema as the presenting feature of familial Mediterranean fever.J Eur Acad Dermatol Venereol. 2013; 27: 912-915Crossref PubMed Scopus (20) Google Scholar Urticaria during attacks suggests diagnosis of other autoinflammatory diseases such as cryopyrin-associated periodic syndrome (CAPS).5Davis M.D.P. van der Hilst J.C.H. Mimickers of urticaria: urticarial vasculitis and autoinflammatory diseases.J Allergy Clin Immunol Pract. 2018; 6: 1162-1170Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar The patients with familial cold autoinflammatory syndrome (FCAS) (the disease at the mild end of the CAPS spectrum) experience attacks of fever and urticaria triggered by cold.6Hoffman H.M. Rosengren S. Boyle D.L. Cho J.Y. Nayar J. Mueller J.L. et al.Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist.Lancet. 2004; 364: 1779-1785Abstract Full Text Full Text PDF PubMed Scopus (464) Google Scholar It is important to note that the attacks are triggered by general cold exposure such as air-conditioned rooms or cool breezes in FCAS and not by direct contact with cold water or objects, which is the case in cold-induced urticaria.6Hoffman H.M. Rosengren S. Boyle D.L. Cho J.Y. Nayar J. Mueller J.L. et al.Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist.Lancet. 2004; 364: 1779-1785Abstract Full Text Full Text PDF PubMed Scopus (464) Google Scholar The ICCT result is negative in FCAS, whereas it is usually positive in cold-induced urticaria.7Wanderer A.A. Hoffman H.M. The spectrum of acquired and familial cold-induced urticaria/urticaria-like syndromes.Immunol Allergy Clin North Am. 2004; 24: 259-286Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar Our patient was experiencing attacks of high fever, abdominal pain, and chest pain, which were typical for FMF, and cold cloth application to reduce fever triggered the cold-induced urticaria. If the details of the history had not been investigated, the differential diagnosis of autoinflammatory diseases would have been challenging in this patient, suggesting a diagnosis of CAPS rather than FMF. The detailed description of rash made us consider the correct diagnosis as co-occurrence of cold-induced urticaria and FMF in this patient, which was confirmed by the ICCT. ReplyThe Journal of Allergy and Clinical Immunology: In PracticeVol. 7Issue 4PreviewWe thank Batu and Vezir for their comments1 on our article.2 We read their case report with great interest. Full-Text PDF