Sweet’s Syndrome – Causes, Symptoms, Treatment, Complications

Sweet’s Syndrome – Causes, Symptoms, Treatment, Complications

What is Sweet’s Syndrome?

Sweet’s syndrome is a simpler name for the medical diagnosis Acute Febrile Neutrophilic Dermatosis. It is also called Gomm Button disease. This disease was first described by Dr Robert Douglas Sweet’s.

Sweet’s syndrome is a relatively rare skin condition characterized by an acute onset of a group of clinical signs and symptoms including fever, tender skin lesions like papules and nodules and an increased number of neutrophils (a type of white blood cells).

Sweet’s syndrome is more commonly seen in females than in males, with a ratio of 4:1. Recurrence can be seen in one-third of the patients. The pattern and appearance of Sweet’s syndrome skin lesions are quite similar to erythema nodosum. Therefore, a biopsy needs to be done to arrive at a definite diagnosis.

Sweet’s Syndrome can Present in the following Types

1. Classical Sweet’s Syndrome (Idiopathic)

Typically affects women in the age group of 30-50 years. Classical Sweet’s syndrome is often associated with pregnancy and inflammatory bowel disease. It may also be seen in children and young adults.

2. Malignancy Associated

This type of Sweet’s syndrome occurs as paraneoplastic variety. Malignancy associated sweet’s syndrome can be associated with acute myeloid leukemia or may represent a skin affection of undiagnosed cancer or recurrence of cancer in patients who have had cancer previously. About 21% of patients of Sweet’s syndrome belong to this variety.

3. Drug Associated

This type of Sweet’s syndrome is often seen in patients who are receiving Granulocyte Colony Stimulating Factor (G-CSF). Certain other drugs associated with Sweet’s syndrome are antibiotics, anti-epileptic drugs, neoplastic drugs and anti-psychotics.

4. Sweet’s Syndrome in Infancy

About 16% cases of Sweet’s syndrome are seen in children. Sweet’s syndrome in children are associated with immune deficiency and higher rates of recurrence.

How Do You Get Sweet’s Syndrome?

Actual cause and method of occurrence of Sweet’s syndrome are not yet clear. Multiple factors work together to cause Sweet’s syndrome. Clinical studies have shown cytokines to be responsible for the development of Sweet’s syndrome.

Sweet’s Syndrome is also Associated with the Following

  1. Cat scratches and insect bites.
  2. Trauma or injury
  3. Sites of an intravenous catheter
  4. Vaccination Sites
  5. After procedures like biopsies
  6. Sunburned skin
  7. After receiving radiation therapy

What are the Causes of Sweet’s Syndrome?

As it is mentioned previously, there may be multiple causes of Sweet’s syndrome. Some postulated causes of this rare clinical condition are:

  1. Bacterial infection is a possibility since there is associated fever and also, the condition has responded well to antibiotics.
  2. The organism Salmonella and Yersinia Enterocolitica is commonly found in intestinal lesions. This has been confirmed by culture growth and serology tests.
  3. The histopathology and course of skin lesions of Sweet’s syndrome suggests a possible hypersensitivity reaction against antigens of bacteria, viruses or against tumors.
  4. Skin cells and circulating cytokines, immune complexes and defensive action of white blood cell types are known to cause the development of inflammation. These cytokines suggest an autoimmune relation to Sweet’s syndrome.
  5. Granulocyte colony stimulating factor in tumors results in malignancy associated Sweet’s syndrome.
  6. Since the higher incidence of the disease is seen in females, hormonal changes may also play a role in the development of Sweet’s syndrome.

Signs and Symptoms of Sweet’s Syndrome:

  1. Fever is the most prominent and troublesome symptom. Fever may be present continuously throughout the period of illness.
  2. Edema is a common clinical finding with fever and skin lesions.
  3. Skin lesions are red, painful, tender and elevated in nature. Skin lesions may be single or multiple. The most common sites are a face, trunk and extremities.
  4. Skin lesions may be papules, macules, pustules or plaques.
  5. There may be edema causing vesicles on the skin.
  6. Skin lesions may be large and fluid filled in malignancy associated Sweet’s syndrome.
  7. The larger skin lesions may tend to ulcerate.
  8. Smaller multiple skin lesions may join to form one huge skin lesion.
  9. The lesions resolve without any scar formation. Skin lesions have a tendency to recur.
  10. Constitutional symptoms like muscle aches, generalized weakness and loss of appetite are also present.
  11. Signs and symptoms of Sweet’s syndrome may be preceded by an upper respiratory tract or a gastrointestinal tract infection.

Complications of Sweet’s Syndrome:

Sweet’s syndrome may present itself in a wide spectrum of diseases and clinical signs and symptoms. If complicated, Sweet’s syndrome can also cause the following clinical conditions to occur

Bones Osteomyelitis, Acute arthritis, Synovitis
Central Nervous System Acute meningitis, encephalitis, brain stem lesions, “neuro-Sweet’s” disease, Guillain-Barre syndrome, paralysis, polyneuropathy and psychiatric symptoms
Ears Nodules, pustules and plaques affecting the middle or inner ear and tympanic membrane
Eyes Conjuctival lesions, hemorrhage, infections of eye, glaucoma, ocular congestion and swelling.
Kidneys Glomerulonephritis, hematuria or polyuria
Intestines Extensive neutrophilic inflammation, infection of the ileus, pan-colitis
Liver Hepatomegaly, liver enzyme abnormalities and neutrophilic inflammation of the liver
Heart Stenosis and infection of the aorta, cardiomegaly, heart failure, vascular dilatation.
Lungs Bronchitis, neutrophilic inflammation of the airways, pulmonary edema.
Mouth Aphthous ulcers, vesicles in the oral mucosa, inflammation and ulceration of the gums, swelling of the tongue, gums and oral mucosa
Muscles Neutrophilic inflammation of tendons, muscles and synovial space around joints
Spleen Splenomegaly

Eye involvement is common in a classical variety of Sweet’s syndrome. Oral lesions are commonly seen in Sweet’s syndrome associated with blood disorders.

Natural Treatment for Sweet’s Syndrome

A classical variant of Sweet’s syndrome, if left untreated, resolves completely within a few weeks to a month without any scar formation. In malignancy-associated type, even after complete cure of cancer, Sweet’s syndrome may still persist in the patient. Recurrence of skin lesions after complete cure of cancer may indicate relapse of malignancy.

In drug-induced type, immediate cessation of offending drug results in spontaneous resolution of skin lesions.
For any kind of clinical condition, treating the cause is as important as a diagnosis. Corticosteroids, topical ointments, potassium iodide, colchicine and antibiotics or anti-inflammatory drugs are often used for medical management. In severe cases, surgical intervention may also be required.

Natural Ways to Manage Sweet’s Syndrome are

  1. Sunscreen – Always apply a deliberate layer of sunscreen overexposedd areas of the body like face, neck and extremities.
  2. Cover up – Always cover up vulnerable areas of your body before going out into the sun.
  3. Almond Oil applied locally is known to relieve skin lesions temporarily.
  4. Alternative Methods of treatment like ayurveda and homoeopathy can be used after proper consultation. Although there are no documented results yet, but these methods can provide a more holistic approach towards this rare condition.
  5. About 30% skin lesions of Sweet’s syndrome are due to injury. So proper wound care is essential as a way to prevent.
  6. Avoid use of any solutions to clean skin lesions
Medically Reviewed By
Dr. Kaushal M. Bhavsar (MBBS, MD)Assistant Professor in Pulmonary Medicine, GMERS Medical College, Ahmedabad