Sweet Syndrome Research Articles

267 RNA-sequencing reveals common interferon-mediated inflammation in VEXAS syndrome, myelodysplasia cutis and Sweet syndrome

267 RNA-sequencing reveals common interferon-mediated inflammation in VEXAS syndrome, myelodysplasia cutis and Sweet syndrome

277 The transcriptome of Sweet syndrome provides novel insights into its pathogenesis and reveals a prominent interferon signature

277 The transcriptome of Sweet syndrome provides novel insights into its pathogenesis and reveals a prominent interferon signature

Vaping—not such a sweet alternative: a case report of bilateral hand Sweet syndrome

Vaping—not such a sweet alternative: a case report of bilateral hand Sweet syndrome

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

Sweet syndrome: an update on clinical aspects, pathophysiology, and treatment.

Sweet syndrome is a neutrophilic dermatosis characterized by an autoinflammatory nature and a sterile neutrophilic infiltrate. It presents with tender, erythematous, edematous papules or plaques, often accompanied by fever. Aim of this review is to summarize the most meaningful aspects of Sweet syndrome, critically discussing old paradigms and novel findings. A search of the English-language literature was conducted using the terms "Sweet syndrome" and "acute febrile neutrophilic dermatosis." MEDLINE (via PubMed) and Web of Science (WOS) databases were consulted up to June 30, 2024. Since its first identification, new clinical and histopathological variants of Sweet syndrome have been described, highlighting its heterogeneity. Additionally, a multitude of clinical conditions have been increasingly reported in association with Sweet syndrome, ranging from malignancies, autoimmune and infectious disorders. The pathogenesis of the disease is unclear and varies according to the associated conditions. One unifying mechanism is the aberrant activation, proliferation, and skin homing of neutrophils. The mainstay of treatment remains systemic corticosteroids; alternatives include colchicine, dapsone, and potassium iodide. Traditional immunosuppressants, biologic agents, and small molecules have also been described as effective in treating Sweet syndrome. Sweet syndrome is a heterogeneous condition with an elusive pathogenesis. Most cases resolve with corticosteroids, but some remain refractory to various therapies, representing an unmet medical need. Recent evidence on the pathomechanisms underlying Sweet syndrome suggests that not only innate but also adaptive immunity might play roles. Further experimental studies are needed and may help identify new therapeutic targets in the future.

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.

Sweet syndrome in patients with and without malignancy: a retrospective study of 66 cases from a tertiary care centre.

Sweet syndrome is a neutrophilic dermatosis that may be associated with malignancy, particularly haematological malignancy. Considering its rarity, the clinical characteristics of Sweet syndrome are still unclear. We aimed to analyse clinicopathological characteristics, treatment, and outcomes of patients with Sweet syndrome according to concurrent malignancy. We retrospectively reviewed patients with Sweet syndrome at the Department of Dermatology from January 2001 to August 2021. We identified 66 patients (median age: 58 years old; 57.6% male) with Sweet syndrome: 24.2% with the classic form, 36.3% with the malignancy-associated form, and 15.1% with the drug-induced form. Idiopathic Sweet syndrome was most common in the non-malignancy group (18.1%). Leukopenia (p=0.008), anaemia (p = 0.004), and thrombocytopenia (p =0.013) were significantly associated with malignancy. No significant difference in histopathology was identified between patients with and without haematological malignancy. Systemic corticosteroids were the most commonly used therapy (n=44, 66.6%). Relapse of Sweet syndrome was more prevalent in the malignancy group. Patients with Sweet syndrome who have laboratory evidence of leukopenia, anaemia and thrombocytopenia should be investigated for malignancy. Sweet syndrome often occurs as a paraneoplastic feature.

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.

Acquired Cutis Laxa Type 2 (Marshall's Syndrome) Associated with Sweet's Syndrome: A Rare Entity.

Acquired Cutis Laxa Type 2 (Marshall's Syndrome) Associated with Sweet's Syndrome: A Rare Entity.

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