Sweet Syndrome Research Articles

Aberrant presentations of erythema nodosum leprosum

Erythema nodosum leprosum (ENL) typically manifests as tender, coppery nodules accompanied by systemic symptoms and visceral involvement. However, rare morphological variants, such as erythema nodosum necroticans, EMF-like ENL, Sweet Syndrome-like ENL, the Lucio phenomenon, and vesiculobullous have also been documented. This study aims to outline the aberrant presentations of ENL and correlating it with possible triggers like COVID infection.Records of Erythema nodosum leprosum patients for the period of 3 years were collected and patients with aberrant presentations were analysed retrospectively in terms of age, sex, onset of ENL episode, number of ENL episodes, spectrum of leprosy, morphology of lesions,systemic complications, bacteriological index, COVID status and treatment given.Nineteen patients were included in the study. ENL necroticans is the commonest presentation followed by ecthyma like ENL and sweet syndrome like ENL. Almost all patients 14/19(73%) has given the history of COVID infection prior to the onset of ENL and has underwent treatment. The mean ENLIST score of COVID infected individuals was 8 compared to mean ENLIST Score of covid non infected individuals which was 5.Aberrant presentations of ENL can frequently be misdiagnosed, especially in patients without a prior diagnosis of leprosy. Thus, it is crucial to maintain a strong suspicion in these cases to avoid delays in diagnosis and the resulting complications.

A case of acute febrile neutrophilic dermatosis characterized with annular pustules

A case of acute febrile neutrophilic dermatosis characterized with annular pustules

Sweet syndrome with multiorgan involvement exacerbated by gilteritinib

Sweet syndrome with multiorgan involvement exacerbated by gilteritinib

Spesolimab Response in a Patient With Steroid-Resistant Sweet Syndrome.

This case report describes a patient with Sweet syndrome whose condition improved with spesolimab after failure of high-dose corticosteroids.

Systemic Sweet Syndrome Recurrence in Lung Allograft: Autopsy Findings from First Reported Case

Abstract Introduction/Objective Sweet syndrome is a non-vasculitic neutrophilic dermatosis characterized by acute, neutrophilic inflammation, most classically in the skin. Sweet syndrome can, however, manifest with systemic organ involvement including the lungs. Pulmonary involvement by Sweet syndrome is exceedingly rare, and its recurrence in a pulmonary allograft is understudied. Methods/Case Report A 43-year-old female with a history of Sweet syndrome with pulmonary involvement developed chronic bronchiolitis obliterans requiring bilateral lung transplantation. Seven years after transplantation, the patient began exhibiting shortness of breath requiring hospitalization. During the patient’s hospital stay, cytomegalovirus viremia and SARS-CoV-2 positivity were detected, and new oral lesions were concerning for a Sweet syndrome flare. Her respiratory status continued to decline requiring escalating ventilatory support. An autopsy limited to the chest and abdomen was performed after she expired. The pleural cavities had extensive adhesions, and both lungs were heavy. Microscopically, the lungs showed extensive, intralumenal acute inflammation of the bronchi and bronchioles with marked chronic inflammation of the submucosa, histologically similar to the patient’s explanted lung specimen. There was also focal endogenous lipoid pneumonia due to bronchiolar obstruction. In addition to the inflammatory findings, multiple infectious agents were identified in the patient’s respiratory tract including SARS-CoV-2, cytomegalovirus, Epstein-Barr virus, Candida glabrata, Klebsiella pneumoniae, and Stenotrophomonas maltophilia. No underlying malignancy was identified. (This patient’s earlier course was reported previously: PMC8411443) Results (if a Case Study enter NA) NA Conclusion Pulmonary involvement in Sweet syndrome can cause significant morbidity, and prior to this case, allograft recurrence of Sweet syndrome had not previously been reported. For transplant patients with a history of pulmonary Sweet syndrome, recurrence should be considered in cases of allograft dysfunction. This case also highlights the importance of thorough microbiological studies in autopsies of patients with systemic inflammatory diseases being treated with extensive immunosuppressive therapies.

Subcutaneous Sweet Syndrome With Vasculitis Features: Case Report and Review.

Subcutaneous Sweet Syndrome (SSS) is a rare variant of Sweet Syndrome characterized by neutrophilic infiltration of subcutaneous adipose tissue without vasculitis. The presence of vasculitis in SSS is uncommon and poses diagnostic challenges. A 38-year-old female presented with a one-year history of recurrent painful erythematous nodules on her limbs and face. Physical examination revealed asymmetrical erythematous patches and tender subcutaneous nodules with central necrotic eschars on the lower limbs. Laboratory tests were unremarkable except for a mildly elevated erythrocyte sedimentation rate. Histopathological analysis showed significant neutrophilic infiltration within the adipose lobules and vascular walls, along with extravasation of red blood cells, indicating vasculitis. The patient responded promptly to systemic corticosteroids; however, symptoms recurred upon tapering, necessitating ongoing steroid therapy. This case underscores the rare occurrence of vasculitis in SSS, expanding the histopathological spectrum of the disease. Literature review suggests that vasculitis in SSS may result from neutrophil-mediated vascular damage rather than immune complex deposition. The recurrent symptoms upon steroid tapering highlight the therapeutic challenges in managing SSS with vasculitis. Recognition of vasculitis in SSS is crucial for accurate diagnosis and effective management. Further research is warranted to elucidate the pathogenesis and develop targeted treatment strategies for SSS with vasculitis.

Clinical features, treatment, and outcome of granulocyte colony stimulating factor-induced sweet syndrome.

Sweet syndrome (SS) is a rare dermatological adverse reaction caused by granulocyte colony-stimulating factor (G-CSF), but the characteristics of G-CSF-induced SS are unclear. This study aims to elucidate the characteristics of G-CSF-induced SS and offer guidance for its prevention and management. We collected relevant case reports of G-CSF-induced SS by searching pertinent databases until June 30, 2024, and synthesized the data for retrospective analysis. A total of fifty patients were analyzed, with a median age of 44 years (1.7-77). The onset of SS occurred between 2 and 90 days post-administration, with a median onset time of 7 days. The predominant cutaneous manifestations included papules/plaques (74.0%), nodules (32.0%), and vesicles/bullae (24.0%). Fever presented in 74.0% of cases, while extra-cutaneous symptoms appeared in 32% of patients. Skin biopsy revealed key findings such as dermal diffuse neutrophil infiltration (97.8%), leukocytoclasis (19.1%), and dermal papillary edema (27.7%). Following both the cessation of G-CSF and systemic corticosteroids treatment, patients showed symptomatic improvement at a median interval of 7 days (2-70). Clinicians should remain vigilant for the risk of SS during G-CSF administration. Skin biopsy plays a crucial role in confirming SS diagnosis. G-CSF-induced SS exhibits a favorable response to corticosteroids, and re-administration of G-CSF should be avoided due to the risk of symptom recurrence.

Giant cellulitis-like Sweet syndrome mimicking cellulitis: a case report

BackgroundSweet syndrome (acute febrile neutrophilic dermatosis) is an uncommon inflammatory disorder characterized by the abrupt appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin. There are various subtypes, such as classical, drug-induced, malignancy-associated, and the less common variant giant cellulitis-like Sweet syndrome. This case is unique due to its presentation of the giant cellulitis-like variant of Sweet syndrome in a patient from Ethiopia. The unusual distribution of the skin lesions and the initial lack of response to antibiotics make this case particularly noteworthy. It underscores the importance of considering Sweet syndrome in differential diagnoses when faced with atypical skin manifestations and ineffective antibiotic treatment. This contribution adds valuable insights to the scientific literature by highlighting the need for heightened awareness of this rare variant and improving diagnostic accuracy in similar clinical scenarios.Case presentationA 60-year-old Ethiopian male patient who presented to the accident and emergency department with a 5-day history of fever, chills, sweating, and rigor accompanied by a reddish skin color change around the anterolateral region of the right chest wall. On physical examination, there were erythematous, indurated tender plaques with ill-defined borders over the right antero- and posterolateral chest wall with extension to the lateral part of the right neck and medial aspect of the right arm. Subsequently, the patient was started on antibiotics, but there was a suboptimal response. Skin biopsy revealed features suggestive of giant cellulitis-like Sweet syndrome. He was then started on steroids, which significantly improved his symptoms.ConclusionA cautious stance is essential when identifying Sweet syndrome in individuals displaying erythematous plaque-like skin lesions in atypical areas of the body with uneven distribution. Such presentation may signal Sweet syndrome rather than a common infection. If conventional treatments, such as antibiotics, fail to resolve symptoms, consider Sweet syndrome as a potential diagnosis. This approach ensures timely and appropriate treatment, preventing treatment delay and misdiagnosis.

Mimickers of erysipelas and cellulitis: A narrative review.

A diffuse erythematous, edematous tender swelling with a distinct border and local rise in temperature suggest a morphological diagnosis of erysipelas-like eruption. While cellulitis and pseudocellulitis have an ill defined tender erythematous plaque. Erysipelas, and cellulitis are not a straightforward diagnosis as multiple cutaneous eruptions mimic erysipelas. It is important to have comprehensive knowledge about the different causes of erysipelas-like eruptions and pseudocellulitis to reach a diagnosis and treat appropriately. Many infectious diseases such as histoplasmosis, leishmaniasis, dermatophytosis, and infusion of chemotherapeutic agents result in erysipelas-like eruptions and pseudocellulitis. Malignancy-related dermatoses such as carcinoma erysipeloides, erysipeloides melanomatosum, and inflammatory dermatoses such as sweet syndrome, well syndrome, and Crohn's disease present as erysipelas-like eruption.

Canine sterile neutrophilic dermatosis (Sweet-like syndrome): A description of 3 cases

Three dogs were presented with a sudden onset of erythematous skin lesions, fever, and various extracutaneous signs, assigned to canine sterile neutrophilic dermatosis by clinical, laboratory, and histopathologic examination. This disease is very rare and comparable to Sweet syndrome in humans. According to the 4 forms of SS, the 1st and 3rd cases in this case report could be classified as classic/idiopathic SS. In this context, the respiratory signs may be a prodromal stage of SS preceding the skin lesions or possibly an infection of the respiratory tract was the trigger for SS. The 2nd case, on the other hand, shows a clear and the 3rd case a possible connection to pathergy as a consequence of a previous surgical treatment (case 2 a tibial plateau levelling osteotomy [TPLO] 20 days previously, case 3 a hemilaminectomy 90 days previously). This way, both may be assigned to the 4th form of SS. In all 3 cases, an adverse drug reaction as a trigger cannot be ruled out with certainty, as medication was used before the diagnosis was made in each case. Besides the most common extracutaneous signs such as fever and neutrophilia, immune-hemolytic anemia occurred in 2 of the patients. As therapeutic options, in addition to the established immunosuppressive drugs such as prednisolone, ciclosporin and azathioprine, oclacitinib is also included in the treatment regimen. In addition, administration of an antiplatelet agent (clopidogrel), which may be interpreted as a lesson learnt from the first of the presented cases, that died of pulmonary thromboembolism. In case 2, all drugs were tapered and eventually stopped over a period of 2.5 years and the patient has been without recurrence for 4 years. Case 3 is recurrence-free under therapy.

Sweet Syndrome Presenting with Features of Cellulitis Shortly after Femoral Angioplasty.

Sweet syndrome, a rare skin condition related to neutrophil infiltration, may be triggered by angioplasty.Sweet syndrome is easily misdiagnosed as infectious conditions such as cellulitis.

48992 Pregnancy-Associated Sweet Syndrome: A Narrative Review

48992 Pregnancy-Associated Sweet Syndrome: A Narrative Review

Clinical Dilemma—Ecthyma Gangrenosum Versus Sweet Syndrome

Abstract Sweet syndrome is associated with malignancy, drugs, and certain infections; its pathogenesis is not well understood. We report a case of a 30-year-old woman with stage IV breast cancer, who presented with worsening respiratory failure and erythematous skin lesions. Biopsy of the skin lesion was consistent with the Sweet syndrome; however, the culture grew Pseudomonas aeruginosa. The lesions healed without scarring when treated with antibiotics and steroids. This is the first case report of classic Sweet syndrome in association with P. aeruginosa.

Drug-Induced Sweet’s Syndrome by Xcopri

Background: Sweet's syndrome (SS) is a relatively rare, self-limiting dermatological condition predominantly affecting women aged 30-50. It is characterized by the sudden onset of erythematous plaques, nodules, or papules on the skin and is often accompanied by fever, leukocytosis, and systemic symptoms. While most SS cases are idiopathic, approximately 5% are drug-induced. Case: This case report describes a 34-year-old female with a medical history significant for epilepsy who presented with diffuse erythematous papules on the bilateral upper extremities. The onset of these symptoms coincided with the initiation of Xcopri (cenobamate tablets) in her medication regimen. Conclusion: To our knowledge, this report highlights the first case of Xcopri-induced SS. We interpret this patient's presentation as a cutaneous response secondary to an adverse drug reaction and aim to emphasize the importance of obtaining a thorough medication history at each clinical consultation.

Hydroxychloroquine-related Sweet's syndrome

Hydroxychloroquine-related Sweet's syndrome

Sweet Syndrome Induced by AFINITOR: Case Report

Sweet Syndrome Induced by AFINITOR: Case Report

Positionally distinct interferon stimulated dermal immune acting fibroblasts promote neutrophil recruitment in Sweet's syndrome.

Sweet's syndrome is a poorly understood inflammatory skin disease characterized by neutrophil infiltration to the dermis. Single-nucleus and bulk transcriptomics of archival clinical samples of Sweet's syndrome revealed a prominent interferon signature in Sweet's syndrome skin that was reduced in tissue from other neutrophilic dermatoses. This signature was observed in different subsets of cells, including fibroblasts that expressed interferon-induced genes. Functionally, this response was supported by analysis of cultured primary human dermal fibroblasts that were observed to highly express neutrophil chemokines in response to activation by type I interferon. Furthermore, single-molecule resolution spatial transcriptomics of skin in Sweet's syndrome identified positionally distinct immune acting fibroblasts that included a CXCL1+ subset proximal to neutrophils and a CXCL12+ subset distal to the neutrophilic infiltrate. This study defines the cellular landscape of neutrophilic dermatoses and suggests dermal immune acting fibroblasts play a role in the pathogenesis of Sweet's syndrome through recognition of type I interferons.

Sweet syndrome in pregnancy: A narrative review.

The aim of this review is to increase obstetrician awareness of pregnancy-associated Sweet syndrome. Patients present with fever, leukocytosis, and skin eruption, which can mimic other infectious or inflammatory conditions, but do not respond to antibiotics. A search using PubMed, EMBASE, and Web of Science Core Collection was conducted to review all reported cases of pregnancy-associated Sweet syndrome, an acute febrile neutrophilic dermatosis occurring during pregnancy or postpartum. A total of 33 episodes among 30 patients were identified, with the majority (54.5% [18]) of cases occurringwithin the second trimester. Among the 30 patients, skin lesions most commonly affected the head and neck (73.3% [22]), with rare oral or ocular involvement. Leukocytosis was the most common laboratory finding, reported in 96.7% [29] of patients, with neutrophil predominance noted in 70.0% [21]. The diagnosis was confirmed for all patients with pathognomonic results of skin biopsies. Of the 27 cases detailing treatment, systemic corticosteroids were most frequently used (19 cases), followed by conservative management (seven cases), and dapsone (one case). The dapsone-treated patient and 15 of the 19 steroid-treated patients experienced resolution, but additional management strategies were required in the remaining four individuals. Spontaneous resolution occurred during pregnancy in six of the seven conservatively managed individuals, with one patient experiencing spontaneous abortion shortly after skin eruption at 10 weeks of gestation. No associated maternal deaths were reported. Obstetric complications of pregnancy-associated Sweet syndrome included endomyometritis, sterile placental abscesses, and abdominal wall necrosis. Delivery of healthy infants occurred in 24 of the 25 cases that presented fetal outcome, which included two infants who underwent medically indicated preterm deliveries.

A case series of sweet syndrome like erythema nodosum leprosum at tertiary care centrein Western India

A case series of sweet syndrome like erythema nodosum leprosum at tertiary care centrein Western India

Skin Manifestations of VEXAS Syndrome and Associated Genotypes

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined genetic disease with an estimated prevalence of 1 in 4269 men older than 50 years and is marked by systemic inflammation, progressive bone marrow failure, and inflammatory cutaneous manifestations. To define the spectrum of cutaneous manifestations in VEXAS syndrome and the association of these findings with clinical, genetic, and histological features. This observational cohort study included data from 112 patients who were diagnosed with VEXAS-defining genetic variants in UBA1 between 2019 and 2023. Data were collected from medical record review or from patients with VEXAS directly evaluated at the National Institutes of Health in Bethesda, Maryland. To define the spectrum of cutaneous manifestations in VEXAS in association with genetic, histological, and other clinical findings. A secondary outcome was cutaneous response to treatment in VEXAS. Among the 112 patients (median [range] age, 69 [39-79] years; 111 [99%] male), skin involvement was common (93 [83%]), and the most frequent presenting feature of disease (68 [61%]). Of 64 histopathologic reports available from 60 patients, predominant skin histopathologic findings were leukocytoclastic vasculitis (23 [36%]), neutrophilic dermatosis (22 [34%]), and perivascular dermatitis (19 [30%]). Distinct pathogenic genetic variants were associated with specific cutaneous manifestations. The p.Met41Leu variant was most frequently associated with neutrophilic dermal infiltrates (14 of 17 patients [82%]), often resembling histiocytoid Sweet syndrome. In contrast, the p.Met41Val variant was associated with vasculitic lesions (11 of 20 patients [55%]) with a mixed leukocytic infiltrate (17 of 20 patients [85%]). Oral prednisone improved skin manifestations in 67 of 73 patients (92%). Patients with VEXAS treated with anakinra frequently developed severe injection-site reactions (12 of 16 [75%]), including ulceration (2 of 12 [17%]) and abscess formation (1 of 12 [8%]). Results of this cohort study show that skin manifestations are a common and early manifestation of VEXAS syndrome. Genetic evaluation for VEXAS should be considered in older male patients with cutaneous vasculitis, neutrophilic dermatoses, or chondritis. Awareness of VEXAS among dermatologists is critical to facilitate early diagnosis.