Idiopathic, Refractory Sweet’s Syndrome Associated with Common Variable Immunodeficiency

a Case Report and Literature Review

Quindelyn S. Cook, Carlton J. Zdanski, Craig N. Burkhart, Paul Googe, Patrick Thompson, Eveline Y. Wu

Research output: Contribution to journalReview article

Abstract

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. Summary: 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.

Original languageEnglish (US)
Article number32
JournalCurrent Allergy and Asthma Reports
Volume19
Issue number6
DOIs
StatePublished - Jun 1 2019

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Sweet Syndrome
Common Variable Immunodeficiency
Immunoglobulins
Autoimmunity
Therapeutics
Agammaglobulinemia
Skin
Immunomodulation
Immunologic Factors

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Pulmonary and Respiratory Medicine

Cite this

Idiopathic, Refractory Sweet’s Syndrome Associated with Common Variable Immunodeficiency : a Case Report and Literature Review. / Cook, Quindelyn S.; Zdanski, Carlton J.; Burkhart, Craig N.; Googe, Paul; Thompson, Patrick; Wu, Eveline Y.

In: Current Allergy and Asthma Reports, Vol. 19, No. 6, 32, 01.06.2019.

Research output: Contribution to journalReview article

Cook, Quindelyn S. ; Zdanski, Carlton J. ; Burkhart, Craig N. ; Googe, Paul ; Thompson, Patrick ; Wu, Eveline Y. / Idiopathic, Refractory Sweet’s Syndrome Associated with Common Variable Immunodeficiency : a Case Report and Literature Review. In: Current Allergy and Asthma Reports. 2019 ; Vol. 19, No. 6.
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