Idiopathic, Refractory Sweet’s Syndrome Associated with Common Variable Immunodeficiency: a Case Report and Literature Review

  • Quindelyn S. CookEmail author
  • Carlton J. Zdanski
  • Craig N. Burkhart
  • Paul B. Googe
  • Patrick Thompson
  • Eveline Y. Wu
Autoimmunity (T. Tarrant, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Autoimmunity


Purpose of Review

Sweet’s syndrome (SS) is classically considered a hypersensitivity reaction often associated with autoimmune disorders and malignancy. SS has also been increasingly reported to occur with immunodeficiencies. We present a case of treatment-refractory, systemic SS as the initial manifestation in a young child with common variable immunodeficiency (CVID). We also review current literature about SS and concurrent immunodeficiencies and autoimmunity in CVID patients.

Recent Findings

Few case reports exist regarding the co-occurrence of Sweet’s syndrome and primary immunodeficiencies. SS is characterized by a pro-inflammatory state with a neutrophil predominance resulting in a spectrum of clinical manifestations. CVID is a multifactorial antibody deficiency that can be associated with autoimmunity, which some studies have proposed to be secondary to altered CD21 expression. SS occurring in patients with CVID has been infrequently reported, and one case study demonstrated improvement of Sweet’s associated skin lesions with immunoglobulin replacement. In our case, the patient had multi-system SS refractory to multiple immunomodulatory therapies. To our knowledge, this is the first report of the effective and safe use of intravenous tocilizumab and oral lenalidomide to treat SS in a child with CVID. Immunoglobulin replacement reduced the frequency of infections and may have contributed to the opportunity to wean the immunosuppressive therapies for Sweet’s syndrome.


Sweet’s syndrome as an initial manifestation of co-occurring immunodeficiencies is rare, and providers need a high index of suspicion. In addition, treatment of SS associated with an immunodeficiency can be a challenge. Treatment with immunoglobulin replacement reduces the frequency of infections, and in some patients with concurrent SS may improve skin lesions and reduce the need for immunomodulator therapy. Further study is necessary to better understand the pathogenesis of CVID in patients with SS and to identify possible biomarkers that predict who with SS are at risk for developing hypogammaglobulinemia.


Neutrophilic dermatoses Sweet’s syndrome Common variable immunodeficiency Primary immunodeficiency Hypogammaglobulinemia 



Acute generalized exanthematic pustulosis


B cell activating factor receptor


Common variable immunodeficiency


Chronic granulomatous disease


Granulocyte-colony stimulating factor


Granulomatous lymphocytic interstitial lung disease


Human immunodeficiency virus


Inducible co-stimulator


Rheumatoid arthritis


Severe combined immunodeficiency


Sweet’s syndrome


Systemic lupus erythematosus


Transmembrane activator calcium-modulator and cyclophilin ligand interactor



We would like to express our gratitude to Dr. Michael Hershfield, Professor of Medicine and Biochemistry at Duke University for his assistance in evaluating our patient for ADA2 deficiency.

Compliance with Ethical Standards

Conflict of Interest

Drs. Cook, Googe, Wu, Zdanski, and Burkhart declare that they have no conflicts of interest.

Human and Animal Rights

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed Consent

Informed consent was obtained from individual(s) discussed in this case report.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Quindelyn S. Cook
    • 1
    Email author
  • Carlton J. Zdanski
    • 2
  • Craig N. Burkhart
    • 3
  • Paul B. Googe
    • 3
  • Patrick Thompson
    • 4
  • Eveline Y. Wu
    • 1
  1. 1.Division of Allergy, Immunology, and Rheumatology, Department of PediatricsUniversity of North Carolina at Chapel HillChapel HillUSA
  2. 2.Department of Otolaryngology/Head and Neck SurgeryUniversity of North Carolina at Chapel HillChapel HillUSA
  3. 3.Department of DermatologyUniversity of North Carolina at Chapel HillChapel HillUSA
  4. 4.Division of Pediatric Hematology-Oncology, Department of PediatricsUniversity of North Carolina at Chapel HillChapel HillUSA

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