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Review
. 2024 Jul 16:15:1425289.
doi: 10.3389/fimmu.2024.1425289. eCollection 2024.

Sweet syndrome associated with moderate leukocyte adhesion deficiency type I: a case report and review of the literature

Yoshine Saito  1 , Anupama Kewalramani  2 , Xiao P Peng  3 , Aimee Magnarelli  4 , Howard M Lederman  4
Affiliations
Review

Sweet syndrome associated with moderate leukocyte adhesion deficiency type I: a case report and review of the literature

Yoshine Saito et al. Front Immunol. .

Abstract

Sweet syndrome is an acute febrile neutrophilic dermatosis characterized by the infiltration of neutrophils into the skin. It may occur idiopathically or be linked to malignancies, inflammatory or autoimmune diseases. Leukocyte adhesion deficiency type I (LAD-I) is an inborn error immunity wherein leukocytes lack adhesion molecules necessary for migration to infection sites due to mutations in the CD18 gene encoding β2 integrins. We present a case of a 16-month-old female initially diagnosed and treated for Sweet syndrome based on histopathological findings with recurrent flare episodes. Subsequent workup revealed LAD-I, making this case the first documented association between Sweet syndrome and LAD-I. Moreover, we reviewed the pertinent literatures detailing the concurrence of neutrophilic dermatosis and immunodeficiency disorders. This case underscores the significance of comprehensive evaluation for Sweet syndrome patients who are refractory to conventional treatments.

Keywords: inborn error of immunity (IEI); leukocyte adhesion deficiency type 1; neutrophilic dermatosis; sweet syndrome; whole exome sequencing.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Left post-auricular mass. Left ear proptosis with an erythematous, tender post-auricular fluctuant mass.
Figure 2
Figure 2
Sweet syndrome skin lesions. Right and left posterior calf subcutaneous nodules with a central necrotic opening.
Figure 3
Figure 3
Histopathologic findings from patient’s right calf biopsy. Biopsy from the right calf lesion revealed dense dermal neutrophilic infiltration (black arrow), increased eosinophils, and perivascular chronic inflammation without evidence of infection.
See this image and copyright information in PMC

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References

    1. Erdem S, Haskologlu S, Haliloglu Y, Celikencir H, Arik E, Keskin O, et al. . Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1. Clin Immunol. (2023) 253:109691. doi: 10.1016/j.clim.2023.109691 - DOI - PubMed
    1. Roos D, van Leeuwen K, Madkaikar M, Kambli PM, Gupta M, Matthews V, et al. . Hematologically important mutations: leukocyte adhesion deficiency (second update). Blood Cells Mol Dis. (2023) 99:102726. doi: 10.1016/j.bcmd.2023.102726 - DOI - PubMed
    1. Opalinska A, Burdacki A, Kwasniak K. Pyoderma gangrenosum with an underlying leukocyte adhesion deficiency type 1 (LAD-1) and pregnancy in the shade of COVID-19 epidemic: a patient and physician experience. Dermatol Ther (Heidelb). (2021) 11:643–53. doi: 10.1007/s13555-021-00507-x - DOI - PMC - PubMed
    1. Almarza-Novoa E, Kasbekar S, Thrasher AJ, Kohn DB, Sevilla J, Nguyen T, et al. . Leukocyte adhesion deficiency-I: a comprehensive review of all published cases. J Allergy Clin Immunol Pract. (2018) 6:1418–20. doi: 10.1016/j.jaip.2017.12.008 - DOI - PubMed
    1. Madkaikar M, Italia K, Gupta M, Chavan S, Mishra A, Rao M, et al. . Molecular characterization of leukocyte adhesion deficiency-I in Indian patients: identification of 9 novel mutations. Blood Cells Mol Dis. (2015) 54:217–23. doi: 10.1111/pai.13990 - DOI - PubMed

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