Acute febrile neutrophilic dermatosis
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Acute febrile neutrophilic dermatosis is a rare inflammatory skin disorder characterized by a fever and a painful rash on the face, neck, arms, legs, and trunk. It is commonly known as the Sweet syndrome.
Sweet syndrome is classified into three categories: classical, malignancy-associated, and drug-induced. A classical sweet syndrome means that the disorder may be associated with other medical conditions such as respiratory infections, gastrointestinal infections, or pregnancy. Malignancy-associated refers to the appearance of Sweet syndrome together with specific kinds of cancer such as acute myeloid leukemia. A drug-induced Sweet syndrome is a reaction to taking certain drugs including granulocyte colony-stimulating factor (G-CSF), NSAIDS, and antibiotics, such as bactrim.
Corticosteroid medications are the most common method of treatment for Sweet syndrome. Signs and symptoms may disappear following treatment. However, there is a chance of recurrence if the underlying cause of Sweet syndrome is left unresolved.
Symptoms
Sweet syndrome can affect anyone. The symptoms may appear suddenly. They could last for weeks, months, or longer depending on the cause. The symptoms may include:
- Fever
- Painful bump or pus-filled blisters on the face, neck, arms, back, or legs
- Rapidly expanding bumps, clusters up to an inch in diameter
- Lesions of the skin and mouth (sores or tumors)
- Discoloration of the skin
- Pain in the muscles and joints, headaches, and exhaustion
- A general sense of being ill
It is recommended to consult a doctor immediately if a rash is rapidly growing in size or symptoms are progressing.
Causes
The exact cause of Sweet syndrome is unknown. It may develop as a side effect of drug, most commonly a treatment that increases the production of white blood cells. It can be also associated with blood cancers or solid tumors, such as breast cancer or colon cancer.
Risk factors
Several factors may affect one’s risk of getting Sweet syndrome, such as:
- Sex: This condition affects more women than men.
- Age: People between the ages of 30 and 60 are more susceptible of getting this disease.
- Cancer: People who have cancer such leukemia, breast and colon cancer are at a higher risk of developing Sweet syndrome.
- Other medical conditions: Sweet syndrome is sometimes associated with inflammatory bowel disease. It may also occur due to respiratory infections. In most cases, the rash occurs after experiencing flu-like symptoms.
- Pregnancy: Pregnancy can trigger Sweet syndrome.
- Drug sensitivity: Several drugs can induce Sweet syndrome such as antibiotics, azathioprine, NSAIDS and granulocyte colony-stimulating factor (G-CSF), which stimulates the body to make neutrophils, a type of immune system cell.
No studies have shown that ethnicity affects one’s risk of developing Sweet syndrome.
Diagnosis
The diagnosis of Sweet syndrome often starts with a physical examination. The doctor, usually a dermatologist, will closely examine the affected area. In addition, the doctor may ask for the patient’s medical history, experienced symptoms and a few tests prior making a conclusive diagnosis. The required tests include:
- Blood tests: The extracted blood is tested for an excess of white blood cells and other blood diseases.
- Skin biopsy: A small sample of the damaged skin is examined to check if it has the characteristics of Sweet syndrome.
Treatment
The treatment for Sweet syndrome depends on the underlying cause of the condition. In some cases, it may resolve on its own without any medical intervention, although it may take longer. Medications such as corticosteroids can potentially expedite the process. These drugs diminish inflammation and suppress immune system function. Corticosteroids protect tissue by reducing amounts of white blood cells and other immune system components that fight infections.
The most common medications are:
- Pills: Most people are treated with systemic corticosteroid medicines such as prednisone. Long-term use might result in adverse effects such as weight gain, sleeplessness, and bone deterioration. This is not recommended if the patient has only few lesions.
- Creams or ointments: These products have skin-thinning properties thus, is recommended to be carefully applied only in the affected skin or isolated lesions.
- Injections: This is not recommended for patients with multiple sores. A small dose of corticosteroid is directly injected into each sore.
In order to prevent relapse, the medication must be taken for a few weeks. Alternatives to corticosteroids include dapsone, potassium iodide, and colchicine. This is generally prescribed to those who have issues with long-term corticosteroid use.
Most people with Sweet syndrome rash and skin lesions recover without scars. Skin discoloration may continue for a few months after the symptoms have subsided. To avoid recurrence of Sweet syndrome, medication must be taken regularly as advised by the doctor.
