Acute typical food protein-induced enterocolitis syndrome triggered by egg white: Two case reports

Main Article Content

Kosei Yamashita
Yuki Okada
Ikuya Sasaki
Sawa Seki
Toshiyuki Takagi
Megumi Okawa
Aiko Honda
Chihiro Kunigami
Mayu Maeda
Taro Kamiya
Takanori Imai

Keywords

egg white, food allergy, food protein-induced enterocolitis syndrome, FPIES, hen’s egg, oral food challenge

Abstract

Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobulin E (IgE)-mediated food allergy characterized by gastrointestinal symptoms such as vomiting and diarrhea. Since 2018, hen’s egg yolk has been the most common trigger for FPIES in Japan, whereas typical egg white (TEW) FPIES remains rare. Herein, we present two cases of acute TEW FPIES in young children. A 17-month-old girl experienced recurrent vomiting and lethargy following egg white ingestion, despite previously tolerating half an egg yolk and 5 g of egg white. Initial tests showed no sensitization markers (egg white-specific IgE < 0.1 kUA/L; ovomucoid [OVM]-specific IgE < 0.1 kUA/L; skin prick test [SPT] negative). Three oral food challenges (OFCs) at 6-month intervals at our hospital all triggered vomiting and lethargy. Procalcitonin and thymus- and activation-regulated chemokine levels remained elevated for 24 hours, and even after 31 months, the patient had not achieved egg white tolerance. A 14-month-old girl presented with vomiting and lethargy after egg white ingestion while on a strict egg yolk- and egg white-free diet. Blood tests again showed no sensitization markers (egg white-specific IgE < 0.1 kUA/L; OVM-specific IgE < 0.1 kUA/L; SPT negative). Two OFCs at 6-month intervals confirmed FPIES, and even after 2 years, the patient had not achieved egg white tolerance. These cases highlight that egg white can trigger FPIES. Thorough medical history taking and diagnostic evaluation, including OFCs, are essential to distinguish FPIES from immediate-type food allergies.

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References

1 Nowak-Węgrzyn A, Chehade M, Groetch ME, Spergel JM, Wood RA, Allen K, et al. International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary—Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol. 2017;139(4):1111–26.e4. 10.1016/j.jaci.2016.12.966

2 Beaudoin M, Mehra A, Wong LSY, Vazquez-Ortiz M, González-Delgado P, Nowak-Wegrzyn A. An algorithm for the diagnosis and treatment of food protein-induced enterocolitis syndrome (FPIES), 2024 update. Allergy. 2025;80(1):362–5. 10.1111/all.16432

3 Katz Y, Goldberg MR, Rajuan N, Cohen A, Leshno M. The prevalence and natural course of food protein-induced enterocolitis syndrome to cow’s milk: A large-scale, prospective population-based study. J Allergy Clin Immunol. 2011;127(3):647–53.e1. 10.1016/j.jaci.2010.12.1105

4 Alonso SB, Ezquiaga JG, Berzal PT, Tardón SD, San José MM, López PA, et al. Food protein-induced enterocolitis syndrome: Increased prevalence of this great unknown-results of the PREVALE study. J Allergy Clin Immunol. 2019;143(1):430–3. 10.1016/j.jaci.2018.08.045

5 Mehr S, Frith K, Barnes EH, Campbell DE, FPIES Study Group. Food protein-induced enterocolitis syndrome in Australia: A population-based study, 2012–2014. J Allergy Clin Immunol. 2017;140(5):1323–30. 10.1016/j.jaci.2017.03.027

6 Gupta RS, Warren CM, Smith BM, Blumenstock JA, Jiang J, Davis MM, et al. The public health impact of parent-reported childhood food allergies in the United States. Pediatrics. 2018;142(6):e20181235. 10.1542/peds.2018-1235

7 Nowak-Wegrzyn A, Warren CM, Brown-Whitehorn T, Cianferoni A, Schultz-Matney F, Gupta RS. Food protein-induced enterocolitis syndrome in the US population-based study. J Allergy Clin Immunol. 2019;144(4):1128–30. 10.1016/j.jaci.2019.06.032

8 Hua A, El-Zataari M, Hudson E, Sanders GM, Schuler CF IV. Evolution of food protein-induced enterocolitis syndrome (FPIES) index trigger foods and subsequent reactions after initial diagnosis. J Allergy Clin Immunol Pract. 2023;11(10):3179–86.e2. 10.1016/j.jaip.2023.06.032

9 Akashi M, Kaburagi S, Kajita N, Morita H. Heterogeneity of food protein-induced enterocolitis syndrome (FPIES). Allergol Int. 2024;73(2):196–205. 10.1016/j.alit.2024.02.001

10 Ullberg J, Fech-Bormann M, Fagerberg UL. Clinical presentation and management of food protein-induced enterocolitis syndrome in 113 Swedish children. Allergy. 2021;76(7):2115–22. 10.1111/all.14784

11 Su KW, Patil SU, Stockbridge JL, Martin VM, Virkud YV, Huang JL, et al. Food aversion and poor weight gain in food protein-induced enterocolitis syndrome: A retrospective study. J Allergy Clin Immunol. 2020;145(5):1430–7.e11. 10.1016/j.jaci.2020.01.001

12 Lee E, Barnes EH, Mehr S, Campbell DE. An exploration of factors associated with food protein-induced enterocolitis syndrome: Birth, infant feeding and food triggers. Pediatr Allergy Immunol. 2021;32(4):742–9. 10.1111/pai.13448

13 Hayashi D, Yoshida K, Akashi M, Kajita N, Tatsumoto C, Ishii T, et al. Differences in characteristics between patients who met or partly met the diagnostic criteria for food protein-induced enterocolitis syndrome (FPIES). J Allergy Clin Immunol Pract. 2024;12(7):1831–9.e1. 10.1016/j.jaip.2024.03.016

14 Akashi M, Hayashi D, Kajita N, Kinoshita M, Ishii T, Tsumura Y, et al. Recent dramatic increase in patients with food protein-induced enterocolitis syndrome (FPIES) provoked by hen’s egg in Japan. J Allergy Clin Immunol Pract. 2022;10(4):1110–2.e2. 10.1016/j.jaip.2021.11.007

15 Anvari S, Ruffner MA, Nowak-Wegrzyn A. Current and future perspectives on the consensus guideline for food protein-induced enterocolitis syndrome (FPIES). Allergol Int. 2024;73(2):188–95. 10.1016/j.alit.2024.01.006

16 Toyama Y, Ishii T, Morita K, Tsumura Y, Takahashi T, Akashi M, et al. Multicenter retrospective study of patients with food protein-induced enterocolitis syndrome provoked by hen’s egg. J Allergy Clin Immunol Pract. 2021;9(1):547–9.e1. 10.1016/j.jaip.2020.09.065

17 Shimomura M, Tanaka H, Meguro T, Kimura M. Three cases of food protein-induced enterocolitis syndrome caused by egg yolk. Allergol Int. 2019;68(1):110–1. 10.1016/j.alit.2018.04.008

18 Nishino M, Sato S, Nagakura KI, Takahashi K, Asaumi T, Ogura K, et al. Food protein-induced enterocolitis syndrome triggered by egg yolk and egg white. Pediatr Allergy Immunol. 2021;32(3):618–21. 10.1111/pai.13406

19 Ebisawa M, Ito K, Fujisawa T. Committee for Japanese Pediatric Guideline for Food Allergy, The Japanese Society of Pediatric Allergy and Clinical Immunology, Japanese Society of Allergology. Japanese guidelines for food allergy 2020. Allergol Int. 2020;69(3):370–86.

20 Kunigami C, Imai T, Yamashita K, Takagi T, Okawa M, Honda A, et al. Relationship between the diagnosis of food protein-induced enterocolitis syndrome and postemetic procalcitonin levels. J Allergy Clin Immunol Glob. 2023;2(4):100156. 10.1016/j.jacig.2023.100156

21 Makita E, Sugawara D, Kuroda S, Itabashi K, Ichihashi K. Usefulness of thymus and activation-regulated chemokine (TARC) for FPIES diagnosis. Pediatr Allergy Immunol. 2022;33(1):e13649. 10.1111/pai.13649

22 Lopes JP, Cox AL, Baker MG, Bunyavanich S, Oriel RC, Sicherer SH, et al. Peanut-induced food protein-induced enterocolitis syndrome (FPIES) in infants with early peanut introduction. J Allergy Clin Immunol Pract. 2021;9(5):2117–9. 10.1016/j.jaip.2020.12.023

23 Fleischer DM, Chan ES, Venter C, Spergel JM, Abrams EM, Stukus D, et al. A consensus approach to the primary prevention of food allergy through nutrition: Guidance from the American Academy of Allergy Asthma & Immunology. J Allergy Clin Immunol Pract; American College of Allergy, Asthma, and Immunology; and the Canadian Society for Allergy and Clinical Immunology. 2021;9(1):22–43.e4. 10.1016/j.jaip.2020.11.002

24 Ierodiakonou D, Garcia-Larsen V, Logan A, Groome A, Cunha S, Chivinge J, et al. Timing of allergenic food introduction to the infant diet and risk of allergic or autoimmune disease: A systematic review and meta-analysis. JAMA. 2016;316(11):1181–92. 10.1001/jama.2016.12623

25 Hua MC, Yao TC, Liao SL, Tsai MH, Lai SH, Chen LC, et al. Introduction of egg white and yolk to infant diets and early childhood atopic dermatitis. Nutrients. 2023;15(6):1379. 10.3390/nu15061379

26 Leonard SA, Pecora V, Fiocchi AG, Nowak-Wegrzyn A. Food protein-induced enterocolitis syndrome: A review of the new guidelines. World Allergy Organ J. 2018;11(1):4. 10.1186/s40413-017-0182-z

27 Watanabe Y, Sakai H, Nihei M, Miura K, Kumaki S. Early tolerance acquisition in hen’s egg yolk-associated food protein-induced enterocolitis syndrome. J Allergy Clin Immunol Pract. 2021;9(5):2120–2.e2. 10.1016/j.jaip.2020.12.053

28 Ruffner MA, Wang KY, Dudley JW, Cianferoni A, Grundmeier RW, Spergel JM, et al. Elevated atopic comorbidity in patients with food protein-induced enterocolitis. J Allergy Clin Immunol Pract. 2020;8(3):1039–46. 10.1016/j.jaip.2019.10.047

29 Ruiz-García M, Díez CE, García SS, del Río PR, Ibáñez MD. Diagnosis and natural history of food protein-induced enterocolitis syndrome in children from a tertiary hospital in central Spain. J Investig Allergol Clin Immunol. 2014;24(5):354–6.

30 Ibrahim T, Argiz L, Infante S, Arasi S, Nurmatov U, Vazquez-Ortiz M. Oral food challenge protocols in food protein-induced enterocolitis syndrome: A systematic review. J Allergy Clin Immunol Pract. 2025;13(4):814–32. S2213-2198(24)01282-0. 10.1016/j.jaip.2024.12.033

31 Kunigami C, Imai T, Yamashita K, Takagi T, Okawa M, Honda A, et al. Procalcitonin level after positive food protein-induced enterocolitis syndrome (FPIES) oral food challenge predicts short-term tolerance. J Allergy Clin Immunol Pract. 2024;12(7):1937–9.e1. 10.1016/j.jaip.2024.03.027