CLINACASE

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ID: 157
Category: Skin Condition
CreatedBy: 1
UpdatedBy: 1
createdon: 10 Feb 2023
updatedon: 05 Apr 2023

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Author: Khoa Tran
Published Feb 10, 2023
Updated Apr 05, 2023

Table of contents

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Etymology and Pronunciation

Acute (ah-kyoot)
acutus - Latin for "sharp"

Febrile (FEB-ril)
febris - Latin for "fever"

Neutrophilic (noo-truh-FIL-ik)
neutrophil - Refers to a type of white blood cell called neutrophils
phílos - Greek for "loving"

Dermatosis (dur-muh-TOH-sis)
derma - Greek for "skin"
-osis - Greek suffix for "pathological" or "disease"

History of Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare inflammatory condition that affects the skin, often presenting as painful red bumps or lesions. The history of Sweet syndrome can be traced back to the early 1960s, when it was first described by Dr. Robert Sweet, a dermatologist from San Francisco.

Dr. Sweet reported on eight cases of a new type of skin disorder in a paper published in the British Journal of Dermatology in 1964. He described the condition as an acute febrile neutrophilic dermatosis, characterized by painful red bumps or plaques on the skin, fever, and an increase in white blood cells called neutrophils.

In the decades following Dr. Sweet's initial report, researchers have made significant progress in understanding the underlying causes and mechanisms of Sweet syndrome. It is now believed to be an autoimmune disorder, in which the body's immune system attacks its own tissues, including the skin.

Sweet syndrome is often associated with other underlying conditions, such as infections, inflammatory bowel disease, and certain types of cancer. Treatment typically involves addressing the underlying condition, as well as the use of corticosteroids or other immunosuppressive medications to reduce inflammation and manage symptoms.

Modern Understanding of Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Sweet Syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare skin condition characterized by skin rash, inflammation, and lesions. The skin lesions are typically raised skin plaques, pus-filled blisters, or ulcerated lesions. It is thought that the condition is an abnormal response of the immune system.

The symptoms of Sweet Syndrome often occur along with fever, leukocytosis (an increase in white blood cells), anemia (a decrease in red blood cells), and various other symptoms. The exact cause of Sweet Syndrome is not fully understood, but it has been linked to a number of underlying health conditions, including inflammatory bowel disease, hematological disorders, and cancer.

In some cases, Sweet syndrome may resolve on its own without treatment.

Sweet Syndrome has also been associated with pregnancy, infections, and certain drugs, such as those used to treat chronic myeloid leukemia, acute myeloid leukemia, myelodysplastic syndrome, and other conditions. In addition, Sweet Syndrome has been linked to a number of inflammatory conditions, including inflammatory arthritis, intestinal tuberculosis, Crohn's disease, ulcerative colitis, systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, granulomatosis with polyangiitis, and vasculitis.

The mechanism behind Sweet Syndrome is not fully understood, but it is thought to involve both acute and chronic inflammation, as well as neutrophil and monocyte activation and increased cytokine production, including interleukin-1 beta.

Sweet syndrome can affect people of all ages and is more common in women than in men. It can occur alone or in association with other medical conditions, such as cancer or autoimmune disorders. Treatment typically involves the use of topical or oral corticosteroids to reduce inflammation and relieve symptoms.

Causes of Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

The exact cause of Acute Febrile Neutrophilic Dermatosis (AFND), also known as Sweet Syndrome, is not fully understood. However, it is believed to be an autoimmune disorder, in which the body's immune system mistakenly attacks its own tissues, including the skin.

Research suggests that AFND may be triggered by various factors, including infections, medication use, and certain underlying medical conditions. In some cases, no underlying cause can be identified.

Infections: Bacterial, viral, and fungal infections have been associated with the development of AFND. Common triggers include upper respiratory infections, gastrointestinal infections, and urinary tract infections.

Medications: Some medications, such as antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), and granulocyte colony-stimulating factor (G-CSF), have been associated with the development of AFND. In some cases, symptoms may resolve once the medication is discontinued.

Underlying medical conditions: AFND may be associated with certain medical conditions, such as inflammatory bowel disease, rheumatoid arthritis, and hematologic malignancies like leukemia.

In some cases, no underlying cause can be identified, and the condition is considered idiopathic.

Regardless of the underlying cause, AFND is characterized by the presence of neutrophils, a type of white blood cell, in the skin. These neutrophils cause inflammation and lead to the formation of painful red bumps or plaques on the skin. Fever and other flu-like symptoms may also occur.

Treatments for Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Treatment for Sweet Syndrome typically involves antibiotics and corticosteroid therapy, as well as other medications to manage the underlying cause of the condition. In some cases, the condition may go away on its own without treatment, but in others, it may persist for months or even years.

Sweet Syndrome is a rare and complex condition, but with proper treatment and management, individuals with this condition can often lead a normal and healthy life. If you think you may have Sweet Syndrome, it is important to talk to your doctor and receive a proper diagnosis and treatment plan.

Symptoms

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Painful small inflamed bumps on your arms, face, neck or back
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Bumps that grow quickly in size, spreading into painful clusters up to an inch (2.5 centimeters) in diameter
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Red spots on skin
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Fever

Confirmation Tests

- Blood test
- Skin biopsy

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