Case Report
 

A Rare Case of Hyper IgE Syndrome with Vocal Cords Involvement

Abstract

Hyperimmunoglobulin E syndrome (HIGE) is considered as a phagocytic or a newly classified complex and heterogeneous primary immunodeficiency disease with symptoms such as increased levels of immunoglobulin E, eczema, and, recurrent lung and skin infections. In this paper, we have presented a rare case of this syndrome. A 9-year-old Iranian girl presented with a history of pruritic maculopapular rash who was eventually diagnosed as a case of HIGE. In her recent admission, she had dysphonia, stridor and huge cauliflower cutaneous lesions on her neck, finger and vocal cords, which did not respond to intravenous antibiotics, and ultimately required surgical removal.

1. Yong PF, Freeman AF, Engelhardt KR, Holland S, Puck JM, Grimbacher B. An update on the hyper-IgE syndromes. Arthritis Res Ther 2012; 14(6):228.

2. Buckley RH, Wray BB, Belmaker EZ. Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics 1972; 49(1):59-70.

3. Freeman AF, Holland SM. The hyper-IgE syndromes. Immunol Allergy Clin North Am 2008; 28(2):277-91.

4. Grimbacher B, Belohradsky BH, Holland SM. Immunoglobulin E in primary immunodeficiency diseases. Allergy 2002; 57(11):995-1007.

5. Grimbacher B, Holland SM, Gallin JI, Greenberg F, Hill SC, Malech HL et al. Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder. N Engl J Med 1999; 340(9):692-702.

6. Freeman AF, Holland SM. Clinical manifestations of hyper IgE syndromes. Dis Markers 2010; 29(3-4):123-30.

7. Renner ED, Puck JM, Holland SM, Schmitt M, Weiss M, Frosch M, et al. Autosomal recessive hyperimmunoglobulin E syndrome: a distinct disease entity. J Pediatr 2004; 144(1):93-9.

8. Szczawinska-Poplonyk A, Kycler Z, Pietrucha B, Heropolitanska-Pliszka E, Breborowicz A, Gerreth K. The hyperimmunoglobulin E syndrome--clinical manifestation diversity in primary immune deficiency. Orphanet J Rare Dis 2011; 6:76.

9. Farmand S, Sundin M. Hyper-IgE syndromes: recent advances in pathogenesis, diagnostics and clinical care. Curr Opin Hematol, 2015. 22(1):12-22.

10. Moin M, Farhoudi A, Movahedi M, Rezaei N, Pourpak Z, Yeganeh M, et al. The clinical and laboratory survey of Iranian patients with hyper-IgE syndrome. Scand J Infect Dis 2006; 38(10):898-903.

Files
IssueVol 18, No 2 (2019) QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/ijaai.v18i2.926
Keywords
Cauliflower Hyperimmunoglobulin E syndrome Immunodeficiency Lesions

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Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Sharafian S, Movahedi M, Kalantari A, Parvaneh N, Gharagozlou M. A Rare Case of Hyper IgE Syndrome with Vocal Cords Involvement. Iran J Allergy Asthma Immunol. 2019;18(2):225-229.