Subcutaneous Sweet’s syndrome: a rare subtype of acute febrile neutrophilic dermatosis
-
Austin B. Ambur
and
Timothy A. Nyckowski
A 58-year-old woman presented to the hospital in July 2021 with a 2 month history of night sweats and a sudden painful cutaneous eruption on the bilateral dorsal forearms. She denied additional systemic symptoms including arthralgia, fatigue, and weight loss. Physical examination was remarkable for multiple edematous and erythematous plaques on the bilateral forearms (Figure 1). A punch biopsy was performed and hematoxylin and eosin staining (H&E) demonstrated a dermal and subcutaneous infiltrate of neutrophils and histiocytoid cells (Figure 2). Laboratory work was remarkable for an elevation of erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP), leukocytosis, neutrophilia, and negative urinalysis. T-cell gene rearrangement was negative, which made subcutaneous panniculitis like T-cell lymphoma unlikely. Importantly, she was up-to-date with her routine cancer screening, including a colonoscopy, mammogram, and pap smear, all of which were unremarkable for malignancy. The clinicopathological findings were most consistent with subcutaneous Sweet’s syndrome (SS) without associated underlying malignancy. A steroid taper was initiated with full resolution over the following month.
Physical examination. (A) Multiple edematous and erythematous plaques on the bilateral forearms. (B) Closer view of an edematous and erythematous plaque on the right forearm.
Pathological examination. (A) The section of skin shows a lymphocytic infiltrate in the superficial and mid dermis (H&E, 10×). (B) The section of skin shows a lymphocytic infiltrate in the superficial and mid dermis. The overlying epidermis demonstrates moderate spongiosis (H&E, 20×). (C) The section of skin shows a dense neutrophilic infiltrate in the dermis. A dense perivascular lymphocytic infiltrate is shown in the superficial dermis (H&E, 100×). (D) A dense perivascular lymphocytic infiltrate is shown in the mid and deep dermis. A dense neutrophilic infiltrate is shown in the deep dermis (H&E, 400×).
SS is an uncommon inflammatory disorder which is characterized by the rapid appearance of painful, edematous, and erythematous cutaneous plaques. Systemic manifestations that may accompany the cutaneous features include fever, leukocytosis, and variable internal organ involvement. The five major subtypes of SS include classic, cancer-associated, inflammatory disease-related, drug-induced, and pregnancy-associated.
The pathogenesis of SS has not been fully elucidated, however it is postulated to be associated with several factors including hypersensitivity reactions, cytokine dysregulation, and a genetic susceptibility [1]. Bacteria, viruses, or tumors may promote the activation and infiltration of neutrophils by stimulating the production of various cytokines [1]. The inflammatory response created by cytokines — including G-CSF, GM-CSF, Il-1, Il-3, Il-6, and Il-8 — may further propagate the inflammatory response seen in SS [1]. Importantly, increased G-CSF production by tumor cells may have a role in malignancy-associated SS [2]. A genetic component may be involved in the susceptibility to SS which may involve abnormalities in chromosome 3q, HLA-B54, or the MEFV gene [1]. Serological testing may demonstrate neutrophilia, elevated ESR, and elevated CRP. Histologic features characteristic of SS are prominent superficial dermal edema with an associated dense neutrophilic infiltrate, leukocytoclasis, endothelial swelling, and an absence of vasculitis. Subcutaneous SS is a unique histological variant which has a strong association with cancer-associated SS [3]. It is characterized histologically by an infiltrate of neutrophils in the subcutis causing a lobular or septal panniculitis and clinically by deep-seated red nodules on the extremities [4]. It is important to highlight that this subtype may mimic other diseases associated with a dermal predominant process, namely erythema nodosum and subcutaneous panniculitis-like T-cell lymphoma [4]. A histological finding of subcutaneous SS should prompt a clinical suspicion for hematologic malignancies, such as acute myelogenous leukemia, and solid tumors of the genitourinary system, breast, and gastrointestinal tract [3]. Corticosteroid therapy is considered first-line treatment. Colchicine, dapsone, and potassium iodide are less frequently used, but may be considered as alternative therapy when a patient is unable to tolerate corticosteroids due to comorbidities. The condition typically resolves within 2–3 months without scarring, however it can recur in up to one-third of patients.
-
Research funding: None reported.
-
Author contributions: Both authors provided substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; both authors drafted the article or revised it critically for important intellectual content; both authors gave final approval of the version of the article to be published; and both authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
-
Competing interests: None reported.
-
Informed consent: The patient described in this report provided written informed consent.
References
1. Villarreal-Villarreal, CD, Ocampo-Candiani, J, Villarreal-Martínez, A. Sweet syndrome: a review and update. Actas Dermosifiliogr 2016;107:369–78. https://doi.org/10.1016/j.ad.2015.12.001.Search in Google Scholar PubMed
2. Shinojima, Y, Toma, Y, Terui, T. Sweet syndrome associated with intrahepatic cholangiocarcinoma producing granulocyte colony-stimulating factor. Br J Dermatol 2006;155:1103–4. https://doi.org/10.1111/j.1365-2133.2006.07521.x.Search in Google Scholar PubMed
3. Nelson, CA, Noe, MH, McMahon, CM, Gowda, A, Wu, B, Ashchyan, HJ, et al.. Sweet syndrome in patients with and without malignancy: a retrospective analysis of 83 patients from a tertiary academic referral center. J Am Acad Dermatol 2018;78:303–9.e4. https://doi.org/10.1016/j.jaad.2017.09.013.Search in Google Scholar PubMed
4. Guhl, G, García-Díez, A. Subcutaneous sweet syndrome. Dermatol Clin 2008;26:541–51. https://doi.org/10.1016/j.det.2008.06.003.Search in Google Scholar PubMed
© 2022 the author(s), published by De Gruyter, Berlin/Boston
This work is licensed under the Creative Commons Attribution 4.0 International License.
Articles in the same Issue
- Frontmatter
- Cardiopulmonary Medicine
- Case Report
- Safe endovascular retrieval of a vena cava filter after duodenal perforation
- General
- Original Article
- Mask-related skin changes among healthcare workers in a community-based hospital
- Medical Education
- Original Article
- The effect of the COVID-19 pandemic on osteopathic education in ACGME-OR residencies from February 2020 to February 2021
- Musculoskeletal Medicine and Pain
- Original Article
- Pain and functional recovery from chronic low back pain over 12 months: implications for osteopathic medicine
- Case Report
- Chronic exertional compartment syndrome in a patient with restrictive cardiomyopathy and portal hypertension
- Public Health and Primary Care
- Original Article
- Casirivimab/imdevimab treatment for outpatient COVID-19 during a SARS-CoV-2 B1.617.2 (Delta) surge at a community hospital
- Clinical Images
- The groove sign
- Acute pericarditis confirmed by coronary computed tomography angiogram
- Subcutaneous Sweet’s syndrome: a rare subtype of acute febrile neutrophilic dermatosis
- Abstracts
- 2022 AOA Research Abstracts and Poster Competition
Articles in the same Issue
- Frontmatter
- Cardiopulmonary Medicine
- Case Report
- Safe endovascular retrieval of a vena cava filter after duodenal perforation
- General
- Original Article
- Mask-related skin changes among healthcare workers in a community-based hospital
- Medical Education
- Original Article
- The effect of the COVID-19 pandemic on osteopathic education in ACGME-OR residencies from February 2020 to February 2021
- Musculoskeletal Medicine and Pain
- Original Article
- Pain and functional recovery from chronic low back pain over 12 months: implications for osteopathic medicine
- Case Report
- Chronic exertional compartment syndrome in a patient with restrictive cardiomyopathy and portal hypertension
- Public Health and Primary Care
- Original Article
- Casirivimab/imdevimab treatment for outpatient COVID-19 during a SARS-CoV-2 B1.617.2 (Delta) surge at a community hospital
- Clinical Images
- The groove sign
- Acute pericarditis confirmed by coronary computed tomography angiogram
- Subcutaneous Sweet’s syndrome: a rare subtype of acute febrile neutrophilic dermatosis
- Abstracts
- 2022 AOA Research Abstracts and Poster Competition
