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  • Author
    Renae Tessem
  • PI

    Philip O. Scumpia, M.D.

  • Co-Author

    Lawrence F. Kuklinski, M.D.; Philip O. Scumpia, M.D.

  • Title

    Neutrophilic Dermatosis following COVID-19 Vaccination: A Case Report

  • Program

    STTP

  • Other Program (if not listed above)

  • Abstract

    Neutrophilic Dermatosis following COVID-19 Vaccination: A Case Report

    Authors: Renae A. Tessem, B.S.; Lawrence F. Kuklinski, M.D.; Philip O. Scumpia, M.D.

    Introduction: Here we present a case of neutrophilic dermatosis of the dorsal hands (NDH) following recent SARS-CoV-2 vaccination in a patient with history significant for chronic lymphocytic leukemia. NDH is a rare, localized variant of Sweet Syndrome (SS) characterized by painful erythematous nodules, a dense neutrophilic infiltrate in the upper dermis generally without vasculitis, elevated neutrophil count, and pyrexia [1, 2].

    Case Description: An XX-year-old male with chronic lymphocytic leukemia presented to the VA dermatology clinic with painful lesions on the dorsal hands bilaterally, of 4 days duration. Review of systems was significant for recent fever, loss of smell, and diarrhea. 4 days prior to the onset of his symptoms, he had received the first dose of an approved vaccine for SARS-CoV-2. No treatments had been tried prior to initial evaluation. Physical examination of the dorsal hands revealed several red to violaceous, juicy, tender papules, some with overlying bullae with an erythematous border. A punch biopsy was obtained showing only minimal inflammation and mild vasculopathy. Thorough laboratory evaluation was unrevealing and mid turbinate COVID-19 PCR testing returned negative twice. Tests for HIV, HBV, ANA, SPEP, UPEP, and IFE also returned negative. Bacterial, fungal, and HSV cultures were negative. Neutrophils were within normal limits (absolute count: 4,490/ml) and unchanged from surveillance labs taken every two months. The patient had a chronically elevated WBC (53,680/ml) and lymphocyte (47,420/ml) count that were within his trend for CLL. His symptoms continued to worsen despite the use of high-potency topical steroids. At 1-week follow-up, physical exam revealed an erythematous juicy plaque with bullae over the right dorsal hand extending to the second finger. On repeat biopsy, histologic findings included a dense neutrophilic infiltrate and superficial dermal edema. Special stains for microorganisms were negative. Based on clinical and histologic presentation, the patient was diagnosed with neutrophilic dermatosis of the dorsal hands, a rare, localized variant of Sweet Syndrome. The patient was started on systemic steroids, which led to rapid improvement. At subsequent follow-up with primary care, the condition had resolved and did not recur following administration of the second dose of the SARS-CoV-2 vaccine.

    Discussion: The patient has known CLL that was deemed stable. Hematologic malignancies are a known risk factor for neutrophilic dermatosis and the patient’s recent vaccination may have been an acute trigger. Classical SS has known associations with underlying hematologic malignancy, inflammatory disease, pregnancy, preceding upper respiratory or gastrointestinal infection, and vaccination [1]. Within the last 50 years, there have been a small handful of reported cases of neutrophilic dermatosis following vaccination for Smallpox, TB, Pneumococcal, and Influenza [3]. Of note, this patient received the Herpes Zoster (Shingles) vaccine, Pneumococcal PPSV23 (Pneumovax), Pneumococcal PCV13 (Prevnar13), TDAP, and the Human Papillomavirus (HPV) vaccine in recent years (2016 or after) with no reported adverse reaction. He was also on chronic acyclovir for frequent bouts/flares of HSV. In the wake of the COVID-19 pandemic, recent publications have reported additional cases of Sweet Syndrome following SARS-CoV-2 vaccination [4] and COVIDÔÇÉ19 infection [5]. Nevertheless, further study is required to understand the pathophysiology of Sweet Syndrome and confirm a causal relationship between SARS-CoV-2 vaccination and the development of neutrophilic dermatosis of the dorsal hands. Finally, it is significant to recognize that NDH is commonly misdiagnosed and treated with antibiotics or surgery [6]. Therefore, increased awareness and understanding of NDH and its associations are vital for accurate diagnosis and rapid administration of highly effective steroid therapy.

    References:

    1. Cohen PR, Kurzrock R. Sweet's syndrome: a neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol. 2000 May-Jun;18(3):265-82. doi: 10.1016/s0738-081x(99)00129-7. PMID: 10856659.
    2. Sweet RD. An Acute Febrile Neutrophilic Dermatosis. Br J Dermatol. 1964 Aug-Sep;76:349-56. doi: 10.1111/j.1365-2133.1964.tb14541.x. PMID: 1420118
    3. Wolf R, Barzilai A, Davidovici B. Neutrophilic dermatosis of the hands after influenza vaccination. Int J Dermatol. 2009 Jan;48(1):66-8. doi: 10.1111/j.1365-4632.2009.03897.x. PMID: 19126054.
    4. Darrigade AS, Théophile H, Sanchez-Pena P, Milpied B, Colbert M, Pedeboscq S, Pistone T, Jullié ML, Seneschal J. Sweet syndrome induced by SARS-CoV-2 Pfizer-BioNTech mRNA vaccine. Allergy. 2021 Jun 18. doi: 10.1111/all.14981. Epub ahead of print. PMID: 34143448.
    5. Conforti C, Dianzani C, Agozzino M, Giuffrida R, Marangi GF, Meo ND, Morariu SH, Persichetti P, Segreto F, Zalaudek I, Neagu N. Cutaneous Manifestations in Confirmed COVID-19 Patients: A Systematic Review. Biology (Basel). 2020 Dec 5;9(12):449. doi: 10.3390/biology9120449. PMID: 33291502; PMCID: PMC7762103.
    6. Wolf R, Tüzün Y. Acral manifestations of Sweet syndrome (neutrophilic dermatosis of the hands). Clin Dermatol. 2017 Jan-Feb;35(1):81-84. doi: 10.1016/j.clindermatol.20109.011. Epub 2016 Sep 12. PMID: 27938816.
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