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ACUTE FEBRILE NEUTROPHILIC DERMATOSIS

Acute febrile neutrophilic dermatosis is also called as a sweet syndrome. It is a rare skin disease occurring more in females than in males. There are three types of sweet syndrome, which include cancer-related, drug-related and unknown cause (idiopathic) sweet syndrome.  The exact cause of the sweet syndrome is unknown, however, in some people it is triggered by an infection, illness or use of medications.

 

The patient may have a fever, swelling in the joints and painful skin lesions on the face, neck, back, and arms. The patient is mainly treated with corticosteroid medications such as prednisone. The signs and symptoms begin to reduce soon after starting the treatment and the patient may recover completely without any skin scars. Recurrence of the disease is very common.

 

SIGNS AND SYMPTOMS

The following are the signs and symptoms of acute febrile neutrophilic dermatosis.

 

  • Painful lesions appear on the arms, neck, head or trunk
  • High to moderate fever
  • Mouth ulcers
  • Joint pains
  • Headache
  • Sore eyes
  • Fatigue

The red color bumps formed on the body may increase in size and form clusters. In some cases, sweet syndrome may also affect bones, liver, heart, lung, kidneys, intestine, mouth muscles, spleen, ears and eyes.

CAUSES

Acute febrile neutrophilic dermatosis is believed to be an autoimmune disorder. It may be triggered by the following factors:

  • Underlying infection, inflammation or illness
  • Solid tumors (breast cancer)
  • Rheumatoid arthritis
  • Immunodeficiency
  • Cancer (leukemia)
  • Ulcerative colitis
  • Sun exposure
  • Vaccinations
  • Medicines

RISK FACTORS

The following are the risk factors of acute febrile neutrophilic dermatosis:

  • Recent recovery from upper respiratory tract infection
  • Health conditions (inflammatory bowel disease)
  • Age (30 to 50 years)
  • Drug sensitivity
  • Female gender
  • Pregnancy
  • Cancer

 

COMPLICATIONS

In a few cases, people with acute febrile neutrophilic dermatosis may develop infection of the skin lesions. In case of cancer-associated with acute febrile neutrophilic dermatosis, the skin eruption may be the first indication of cancer. The pigmentation on the skin may be reduced within few months.

DIAGNOSIS

The diagnosis of acute febrile neutrophilic dermatosis is based on the presence of following symptoms:

Major symptoms

  • Sudden onset of painful red plaques with or without pustules and vesicles.
  • Presence of neutrophil infiltration in dermis and absence of leukocytoclastic vasculitis (hypersensitivity vasculitis)

Minor symptoms

  • History of fever and discomfort
  • Lab test results showing significant changes
  • Medical history of respiratory tract infection, solid tumor or pregnancy
  • Responding to the treatment of systemic corticosteroids and potassium iodide

 

Laboratory tests

The diagnosis of acute febrile neutrophilic dermatosis can be made with the help of following tests:

  • White blood cell count
  • Neutrophil count
  • Erythrocyte sedimentation rate
  • C-reactive protein

The blood sample from the patient is collected and the above listed tests are done.

It is a technique where sample tissue is taken from the patient and sent to a laboratory for microscopic examination to identify cause, extent, type and presence of disease. The diagnosis of acute febrile neutrophilic dermatosis is confirmed by skin biopsy.

TREATMENT

Acute febrile neutrophilic dermatosis may resolve by itself. However, treatment is based on severity of the patient’s condition.

The patient with few lesions is treated with oral corticosteroid. The long term use of corticosteroid can cause side effects like weight gain, fluid retention, high blood pressure.

Creams and lotions containing corticosteroids are applied directly to the skin lesions. Creams are helpful to treat small skin lesions.

The corticosteroid is injected directly into the skin lesions or in to the affected joint such as knee joint.

 

Acute febrile neutrophilic dermatosis is also treated with other agents like dapsone, potassium iodide, colchicines.

 

PREVENTION

Recurrence of acute febrile neutrophilic dermatosis can be prevented by identifying and treating the underlying disorders. It can also be prevented by protecting the skin from sun exposure by following ways:

  • Applying sunscreen with a sun protection factor (SPF) not less than 15
  • Wearing sunglasses, long-sleeved shirts, and hats
  • Avoiding exposure to midday sunlight
  • Staying indoors 
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