Sweet Syndrome Research Articles

Oral manifestations of COVID-19: A review

An important indicator of general health, well-being, and quality of life is oral health. The SARS-CoV-2 virus, which has been discovered to have a number of adverse effects. One of the earliest tissue areas to become infected by the virus and undergo alterations is the oral cavity. Oral manifestations included ulcer, erosion, bulla, vesicle, pustule, fissured or depapillated tongue, macule, papule, plaque, pigmentation, halitosis, white patches, haemorrhagic crust, necrosis, petechiae, swelling, erythema, and spontaneous bleeding. The tongue (38%), labial mucosa (26%), and palate (22%) were the three most typical sites of involvement. Aphthous stomatitis, herpetiform lesions, candidiasis, vasculitis, mucositis, drug eruption, necrotizing periodontal disease, angina bullosa-like, angular cheilitis, atypical sweet syndrome, and Melkerson-Rosenthal syndrome were suggested diagnoses for the lesions. In 68% of instances, oral lesions were symptomatic. There were almost equally as many oral lesions in both sexes (49% female and 51% male). More extensive and severe oral lesions were present in patients who were older and who had COVID-19 diseases that were more severe. The most significant risk factors for the development of oral lesions in COVID-19 patients include poor oral hygiene, opportunistic infections, stress, immunosuppression, vasculitis, and hyper-inflammatory response. It is crucial to identify any changes in the mucosa in COVID-19 patients and administer assertive treatment to prevent complications. Patients should also try to maintain adequate oral hygiene throughout the course of the illness to prevent the colonisation of opportunistic microorganisms and to prevent complications both orally and systemically.

Drug-induced acute febrile neutrophilic dermatosis (Sweet syndrome): A case report presented at Delhi State Cancer Institute

ABSTRACT Sweet syndrome is a rare, etiologically unknown dermatosis that can be idiopathic or associated with certain clinical conditions. Among these conditions are infections, autoimmune diseases, inflammatory bowel diseases, vaccinations, the use of medications, and neoplasias. Hematological neoplasias, particularly acute myeloid leukemia, are the most commonly described; however, the condition may also be related to solid tumors, being those of the genitourinary tract the most associated with the syndrome. Drug-induced Sweet syndrome has also been reported; however, it is rarely seen. We report a case of 59 years old male patient with a diagnosis of carcinoma urinary bladder, who developed skin eruption after infusion of the first cycle of chemotherapy (gemcitabine and cisplatin) and resolved after treatment with steroids. The diagnosis of Sweet syndrome was confirmed only after skin biopsy, cultures and laboratory investigations. Malignancy as the cause of skin eruptions was eliminated by the fact that it occurred only after infusion of chemotherapy and the tumor was still there in the bladder, and skin eruptions did not occur after stopping steroids.

1548 Identification of a neutrophil-specific PIK3R1 mutation facilitates targeted treatment in a patient with sweet syndrome

1548 Identification of a neutrophil-specific PIK3R1 mutation facilitates targeted treatment in a patient with sweet syndrome

009 The transcriptome of Sweet syndrome suggests a pathogenic role of both innate and adaptive immune signaling pathways

009 The transcriptome of Sweet syndrome suggests a pathogenic role of both innate and adaptive immune signaling pathways

Sweet syndrome associated with malignant disease.

A 45-year-old woman with relapsed acute myeloid leukemia and who was receiving palliative chemotherapy with monthly decitabine was admitted to the oncology ward with a 2-day history of fever (38.5°C) and painful skin lesions on her hands, arms and upper body. The lesions were red to violaceous

Rare cas de syndrome de sweet bulleux induit par produit de contraste ionisé

To our knowledge, sweet bullous syndrome has been associated with contrast media only once in the literature, which makes our case very rare. Our patient developed, 48h after the realization of an injected scanner, a bullous eruption, cocardoides lesions and a fronto-malar erythema. The skin biopsy concluded to a bullous Sweet syndrome. The patient was treated with topical corticosteroids and the lesions disappeared after 7 days. Drug-induced sweet syndrome can mimic several other diagnoses. In our case, the malar erythema is reminiscent of lupus. Drug-induced sweet is a drug hypersensitivity reaction characterized by the following clinical signs: chronological development of a skin lesion following drug exposure, fever of 38.4°C, neutrophil infiltration in the dermis without vasculitis, and resolution of symptoms following steroid use, all of which were present in our case. We report a case of bullous sweet syndrome, an aggressive form which resolved with steroids. Key words: Delayed hypersensitivity; Sweet; Bullous sweet variant; Iodinated contrast media

Neutrophilic dermatoses - Pathomechanistic concepts and therapeutic developments.

Neutrophilic dermatoses are a group of clinically heterogeneous diseases characterized by infiltration of neutrophils in the affected tissue. Skin symptoms comprise a spectrum of wheals, papules, plaques, pustules, nodules and ulcerations often in combination with systemic symptoms. Although the pathogenesis of these diseases has not yet been elucidated in depth, broad pathophysiological and clinical overlaps exist with autoinflammatory syndromes. Additionally, recent years have shown the relevance of the signaling pathways of TNF-α, IL-1, IL-12/23 and IL-17 in neutrophilic dermatoses. In this review, we present four selected neutrophilic dermatoses, namely pyoderma gangraenosum, Sweet syndrome, generalized pustular psoriasis and Schnitzler syndrome, discuss pathophysiological aspects and specifically address novel therapeutic options derived from the most recent pathophysiological findings.

Drug-Induced Subcutaneous Sweet Syndrome

Drug-Induced Subcutaneous Sweet Syndrome

Concurrent Sweet syndrome and erythema nodosum: two sides of the same coin?

Concurrent Sweet syndrome and erythema nodosum: two sides of the same coin?

Acute neutrophilic dermatitis in a neutropenic patient – An uncommon presentation of sweet syndrome

Sweet syndrome (SS) is an uncommon acute febrile neutrophilic dermatosis. Patients present with acute febrile illness, neutrophilia, and tender skin lesions. It is subclassified into idiopathic, malignancy associated, and drug induced. Occasionally neutrophilia may be absent in patients who are neutropenic secondary to malignancy, drugs, and congenital neutropenia. We describe a 70-year-old gentleman presenting with febrile neutropenia and SS. We have also reviewed the previously described reports of neutropenia and SS form literature.

Trimethoprim - Sulfamethoxazole Induced Sweet Syndrome: A Case Report

Sweet syndrome (SS) also called as acute febrile neutrophilic dermatosis, is a rare inflammatory infectious skin reaction characterized clinically by tender, erythematous papules or plaque commonly appearing on the upper limbs, trunk, and head and neck. It is usually accompanied by fever and an elevation in the erythrocyte sedimentation rate (ESR), C- reactive protein. SS is classically triggered by infections, inflammatory bowel disease, pregnancy, malignancy or due to the administration of an associated drugs. Drug induce sweet syndrome (DISS) an uncommon subtype of SS. Drugs like antiepileptics like carbamazepine, diazepam, antibiotics like trimethoprim-sulfamethoxazole (TMP-SMX), minocycline, nitrofurantoin, norfloxacin, ofloxacin, contraceptives, non-steroidal anti-inflammatory drugs (NSAIDS) (celecoxib and diclofenac) and granulocyte – colony stimulating factor are the most common culprit. In this case, the patient was presented with oedematous and pustular eruption of nose with a rapid extension to the face, neck, upper trunk and the limbs which developed after taking TMP-SMX for 10 days accompanied with fever (42ºC) and cough. Laboratory test disclosed elevated WBC with notable increase in polymorphonuclear cells ,elevation of ESR (96mm/h), C-reactive protein (85ng/l, normal <5) and α-2-globulin (10g/l, normal <8). All these presentations clearly indicated that the patient is having DISS as per Walker and Cohen criteria for SS. TMP-SMX was discontinued upon which the lesions started to disappear. Clinical pharmacist must be more aware about the usage of TMP-SMX especially in immune compromised patients. Keywords: Drug induced sweet syndrome, Trimethoprim-sulfamethoxazole, Neutrophilic infiltration, Corticosteroids, oedematous papules,

Skin Biopsies in Gastrointestinal Diseases: A Single Centre Experience from North India

Skin manifestations have always been alarming to the patient and often warrant complete clinical evaluation. A thorough understanding of relationship between gastrointestinal (GI) disorders and skin diseases can alert the clinician to an underlying occult GI disorder. The aim of the study was to review the cutaneous changes in patients with co-existent, often masked GI diseases and to observe the underlying pathophysiology of such lesions. This was a retrospective, record based observational study. The archived histopathology reports & medical records of patients over a period of 5 years were searched and all patients who underwent skin biopsy with co-existent GI diseases were included. Out of a total of 218 patients of GI diseases who had dermatological manifestations, only 44 were biopsied. Pruritis (n=175), koilonychia (n=52) and erythematous rash (n=35) were the most common clinical features. Psoriasis (n=4), vasculitis (n=4), Dermatitis Herpetiformis (n=3) and pyoderma gangrenosum (n=5) were often seen associated with benign GI diseases like Inflammatory Bowel Diseases, Chronic Liver Disease, Malabsorption syndrome etc. Sweets syndrome (n=2) and cutaneous metastatic nodules (n=3) were associated with underlying malignancy particularly of gall bladder and pancreas.

Sweet Syndrome Associated with Myelodysplastic Syndrome—A Review of a Multidisciplinary Approach

Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients.

Acute Onset of Bullous Skin Rash and Acute Kidney Injury after Exposure to Radiocontrast: Sweet's Syndrome.

Patients with Sweet's syndrome (SS) typically present with acute-onset fever, leucocytosis, and erythematous, tender plaques with dense neutrophilic infiltration in the dermis. The condition is classified into three subtypes: classic SS, malignancy-associated SS, and drug-induced SS.Drug-induced SS is characterized by an abrupt onset of a painful erythematous rash, dense neutrophilic dermal infiltrate without vasculitis, a temporal relationship between exposure and onset, and resolution of symptoms after drug discontinuation and/or corticosteroid therapy.Treatment options include systemic corticosteroids as first-line therapy, while colchicine, dapsone, indomethacin, naproxen, clofazimine, ciclosporin, α-interferon, and potassium iodide may be considered as second-line therapies in cases resistant to corticosteroids.

Elevated Intraocular Pressure in Periorbital Sweet's Syndrome.

A 66-year-old immunocompromised man presented with cellulitis around the left eye that was initially concerning for necrotizing fasciitis. Exam findings were remarkable for exquisite periocular tenderness with rigid, immobile eyelids resulting from severe erythema, edema, and induration. Given the concern for orbital compartment syndrome and a necrotizing infection, the patient was taken urgently to the operating room for debridement of the eyelid skin as well as an urgent lateral canthotomy and cantholysis. His eye exam revealed 360° of hemorrhagic chemosis, no relative afferent pupillary defect, and an ipsilateral elevated intraocular pressure of 35 mm Hg. No visual acuity measurement could be obtained secondary to the patient's altered mental status. His intraocular pressure normalized after treatment with antihypertensive drops and further extension of the canthotomy. Histopathological analysis showed extensive neutrophilic infiltrate of the dermis which was compatible with a diagnosis of Sweet's syndrome.

VEXAS syndrome mimicking lupus-like disease.

Dear Editor, We report on an 81-year-old male Caucasian patient, who initially presented with oral ulcerations and disseminated erythematous papules on the upper back, chest, arms and legs with histopathological evidence of lymphocytic infiltration and small vessel vasculitis matching subacute lupus erythematous (Fig. 1A and B). His skin manifestations improved with topical CS and HCQ at standard dosages. However, a complete remission of the lesions could not be achieved. Therefore, repeated skin biopsies were performed, showing features of subacute eczema or Sweet Syndrome. Fourteen months later, the patient developed recurrent episodes of oral ulcerations, painful auricular and nasal erythematous swelling and arthritis in hands and feet (Fig. 1C and D). A hand MRI showed a moderate to severe wrist joint synovitis. Another 6 months later, he presented with clinical symptoms and laboratory findings of anaemia with increased, corpuscular volume (102.9 fl; normal range 81–98 fl), leukopoenia, lymphopenia, thrombocytopenia, increased levels of CRP, serum amyloid A and IL-6 as well as anti-mutated citrullinated vimentin (MCV) antibodies (Fig. 1G). Treatment with MTX was started at 15 mg/week and was well tolerated, leading to improvement of skin and joint symptoms. The patient experienced one episode of epilepsy with generalized convulsions. Cerebral MRI scan showed unspecific cerebral microangiopathy but no specific lesion. To rule out further foci of inflammation, an 18-fluorodeoxyglucose (18FDG)-PET/CT was performed, showing an enhanced FDG uptake typical for bone marrow activation (Fig. 1E and F), in accordance with a previous description [1]. Over the last 6 months, his skin and joints were well controlled by MTX. However, haematological and inflammatory parameters did not improve. As the patient fulfilled the major diagnostic traits of VEXAS [2], including male sex, increased corpuscular volume >100 fl and low platelets G/l, genetic testing was done. This patient was identified to carry the pathogenic variant c.121A>C, p. (Met41Leu) in exon 2 of UBA1 (NM_003334) (ACMG class 5) in mosaic state (68% of 93 reads), confirming the diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome.

Unusual Adverse Events in a Patient With BRAF-Mutated Non–Small Cell Lung Cancer Treated With BRAF/MEK Inhibition

BRAF/MEK inhibition remains standard of care for treatment of BRAF-mutated non-small cell lung cancer (NSCLC). Although common adverse events (AEs) have been reported through clinical trials and ongoing clinical practice, only a handful of reports have detailed unusual adverse events associated with these medications. This report presents a patient with BRAF-mutated NSCLC treated with dabrafenib and trametinib who experienced 2 unusual AEs-Sweet syndrome and MEK-associated retinopathy-that responded to steroid treatment. The patient was able to continue BRAF/MEK inhibition through a coordinated multidisciplinary approach. This case highlights the importance for all clinicians to recognize unusual AEs associated with BRAF/MEK inhibition, particularly in the setting of expanded use for all BRAF V600E-mutated solid tumors.

Diagnostic Criteria in Dermatology

Diagnostic criteria are defined as group of features that can collectively be used to diagnose a condition. Diagnostic criteria are used for diagnosing those skin conditions that have heterogenous presentation and do not have a single specific clinical, laboratory or pathological feature. Keywords: Diagnostic criteria, Atopic Dermatitis, Sweets Syndrome, Pyoderma Gangrenosum

Sweet and Wells syndrome: One disease with different cellular infiltrates?

Sweet and Wells syndrome: One disease with different cellular infiltrates?

Lidocaine Induced Laryngeal Angioedema in Sweet Syndrome: Is it incidental or anticipated?

The online version contains supplementary material available at 10.1007/s12070-022-03396-4.