What is Sweet’s syndrome?
Sweet’s syndrome is a rare autoinflammatory condition and form of neutrophilic dermatosis that mainly affects adults, but can also affect children and babies. The main symptoms include fever and painful skin lesions that most often appear on the face, neck and upper extremities, but can appear on any part of the body. On rare occasions, there are no skin lesions. Other common symptoms include fatigue (a medical term that includes a number of different symptoms and isn’t the same as simply feeling tired); muscle pain; joint pain (arthralgia); joint pain and swelling (arthritis); headaches or migraines; eye problems; mouth ulcers. On rare occasions, the lesions can develop in internal joints and organs causing more serious symptoms. Very rarely, SS may be life-threatening. The cause of SS is poorly understood, but it is associated with infections; cancer, particularly blood cancer (malignancy-associated or paraneoplastic); autoimmune conditions; inflammatory bowel disease; medications (drug-induced); pregnancy; skin damage; immunodeficiency; overexposure to sunlight or ultraviolet light; vaccination. In regards to the latter, SS caused by vaccination is very rare – only 11 cases reported in medical literature in the past 44 years, globally.
‘Sweet’s syndrome (also known as acute febrile neutrophilic dermatosis) is a rare skin disorder characterised by a fever and the appearance of tender solid red lumps on the skin. It is a reactive condition with a number of potential triggers. It is not contagious and is not skin cancer.’
‘Acute febrile neutrophilic dermatosis is an uncommon skin condition characterised by fever and inflamed or blistered skin and mucosal lesions. Neutrophilic dermatoses are autoinflammatory conditions often associated with systemic disease.
Acute febrile neutrophilic dermatosis also has the eponymous name, Sweet syndrome or disease—named after Dr Robert Douglas Sweet from Plymouth, England, who first described it in 1964.’
‘Sweet’s syndrome is a rare skin condition. Its main signs include fever and painful skin lesions that appear mostly on your arms, neck, head and trunk.
The exact cause of Sweet’s syndrome isn’t known. In some people, it’s triggered by an infection, illness or certain medications. Sweet’s syndrome can also occur with some types of cancer.’
‘Sweet syndrome is a rare disorder characterized by fever and the sudden onset of a rash, which consists of multiple tender, red or bluish-red bumps or lesions. These lesions usually occur on the arms, legs, trunk, face or neck. In some cases, additional systems of the body can become involved including the musculoskeletal system such as inflammation of the joints (arthritis), the eyes such as inflammation of the conjunctiva or the membrane that lines the eyes (conjunctivitis), and the internal organs. In the majority of affected individuals, the disorder occurs by itself for no known reason (idiopathic Sweet syndrome); this is also known as classical Sweet syndrome. Less often, the disorder can be associated with an underlying cancer (malignancy), usually a blood (hematologic) cancer such as certain types of leukemia; this is known as malignancy-associated Sweet syndrome. The disorder can also result as a reaction to taking certain drugs, especially a drug known as granulocyte-colony stimulating factor; this is known as drug-induced Sweet syndrome. Sweet syndrome is treated with corticosteroids.’
‘Sweet’s syndrome is a disorder of neutrophils (pus cells) and although it is not considered hereditary, there is some evidence of a genetic link.
Most experts consider Sweet’s syndrome a ‘reactive condition’ – it is driven by an underlying condition in up to 50% of cases. As the appearance of Sweet’s syndrome may occur before the underlying condition is recognised, ongoing monitoring is required, especially if the skin eruption is severe, recurrent or treatment-resistant or if blood abnormalities are detected.’
‘Sweet’s syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Several hundreds cases of Sweet’s syndrome have been published. Sweet’s syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced.’
‘Sweet’s syndrome is a neutrophilic dermatosis, characterised by fever, peripheral neutrophil leucocytosis and the acute onset of painful, erythematous papules, nodules or plaques.’
1 current trial: Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases (NEUTROGENE).
Provides a summary and coding information. ICD-10: L98.2
2012-2018 Sweet’s Syndrome UK