sweet syndrome

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a condition primarily affecting the skin and is characterized by a sudden onset of fever along with painful, red lesions such as papules, nodules, and plaques. The condition is marked by a dense, predominantly neutrophilic infiltrate in the upper dermis and is associated with systemic inflammation. Genetic factors appear to contribute, with variants in the MEFV gene implicated, and the disorder follows an autosomal dominant inheritance pattern. Although precise prevalence estimates are not well established, patients typically present with rapid-onset skin and systemic symptoms that prompt further investigation.

The clinical presentation of sweet syndrome is dominated by inflammatory skin findings and systemic symptoms. Nearly all affected individuals experience arthralgia, myalgia, and cystic acne, while many also present with skin nodules, recurrent fever, and erythematous papules and plaques. Additional laboratory findings often include an elevated circulating C-reactive protein concentration and other markers of inflammation, such as an increased erythrocyte sedimentation rate, supporting the inflammatory nature of the condition. Not all individuals experience all features, and severity varies considerably.

Sweet syndrome is associated with variations in the MEFV gene, which plays a role in regulating the body’s inflammatory response. Disruption in this gene may lead to uncontrolled inflammation, manifesting as the characteristic skin lesions and systemic symptoms seen in the condition. Inheritance is autosomal dominant, meaning that having one altered copy of the gene can contribute to disease development, and there is a potential for the variant to be transmitted to offspring. Due to variable expressivity, family members with the same genetic change may experience differences in the type and severity of symptoms, making genetic counseling a useful resource for affected families.

Currently, management of sweet syndrome focuses on supportive care and symptom control as there are no treatments that have received FDA approval specifically for this condition. Treatment is tailored to each individual and typically involves a multidisciplinary approach, with regular surveillance and ongoing monitoring by healthcare professionals to promptly identify and manage any complications. Patients are encouraged to discuss treatment options with their healthcare team, and genetic counseling is recommended to help families understand the implications of the autosomal dominant inheritance pattern.

The long-term outlook for individuals with sweet syndrome is highly variable, with outcomes largely depending on the severity of symptoms, age at diagnosis, and access to appropriate care. Modern standards of care, including regular surveillance and supportive management, have improved the ability to control symptoms and enhance quality of life. While some patients may experience periods of flare-ups, others find that symptoms stabilize over time, and overall progress can differ significantly between individuals. As outcomes continue to evolve with advances in medical care, families are encouraged to connect with specialists familiar with this condition for ongoing support and guidance.

Clinical trials are underway exploring new treatment approaches for sweet syndrome. Individuals interested in clinical trial opportunities are encouraged to search ClinicalTrials.gov or consult their care team about eligibility and emerging therapies.