Thursday, January 19, 2006
Sweet's Syndrome
Sweet's disease was named after a Dr Sweet from Plymouth, England, who first described this condition in 1964. It is also known as acute neutrophilic dermatosis.
In Sweet's disease there is a sudden appearance of red tender lumps (plaques) on the skin usually of the limbs or neck, sometimes elsewhere. It most often occurs in middle-aged women, but men, children and the elderly may also be affected.
Sweet's is a reaction to an internal condition.
It may follow:
Upper respiratory tract infection (e.g. chest infection, streptococcal throat infection)
Vaccination
Inflammatory bowel disease (e.g. ulcerative colitis or Crohn's disease)
Rheumatoid arthritis
Blood disorders including leukaemia (most often acute myelogenous leukaemia).
Internal cancer usually of bowel, genitourinary organ or breast
Pregnancy
Drugs, including granulocyte colony stimulating factor (G-CSF), nonsteroidal anti-inflammatory medications, cotrimoxasole and several others
In some people, no underlying condition is found.
Sweet's disease generally results in the following symptoms:
High or moderate fever
Tiredness and malaise (feeling unwell)
One or more tender red papules or plaques. These enlarge and persist for several weeks. They may have blisters, pustules or ulcers. Sometimes they appear to clear in the centre.
Aching joints and headache
Sore eyes and/or mouth ulcers
Sometimes other organs are affected including bones, nervous sytem, kidneys, intestines, liver, heart, lungs, muscles and spleen.
Skin lesions may arise at the site of injury such as a needle prick, biopsy or insect bite. It some patients they arise only in sun exposed areas.
Sweet's disease is related to pyoderma gangrenosum, and is sometimes difficult to distinguish from it.
In one variant of Sweet's disease, ‘neutrophilic dermatosis of the dorsal hands’, bluish or grey abscess-like nodules arise on the backs of the hands. These nodules may ulcerate. This is sometimes known as ‘pustular vasculitis’ of the hands, because inflamed blood vessels are seen on biopsy as well as an infiltration of neutrophil white cells.
Investigations may reveal:
Raised erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), indicating systemic inflammatory disease
Raised white cell count (neutrophil leukocytosis)
Numerous neutrophil inflammatory cells on skin biopsy associated with broken-up neutrophils (leukocytoclasia) and swelling of cells lining blood vessels (endothelial cells)
Sweets' lesions resolve eventually without leaving a mark or scar, with or without treatment.
Generally there is a single episode of Sweet's disease, but a third of patients may develop recurrent episodes. This is more likely in patients who have underlyng cancer.
In Sweet's disease there is a sudden appearance of red tender lumps (plaques) on the skin usually of the limbs or neck, sometimes elsewhere. It most often occurs in middle-aged women, but men, children and the elderly may also be affected.
Sweet's is a reaction to an internal condition.
It may follow:
Upper respiratory tract infection (e.g. chest infection, streptococcal throat infection)
Vaccination
Inflammatory bowel disease (e.g. ulcerative colitis or Crohn's disease)
Rheumatoid arthritis
Blood disorders including leukaemia (most often acute myelogenous leukaemia).
Internal cancer usually of bowel, genitourinary organ or breast
Pregnancy
Drugs, including granulocyte colony stimulating factor (G-CSF), nonsteroidal anti-inflammatory medications, cotrimoxasole and several others
In some people, no underlying condition is found.
Sweet's disease generally results in the following symptoms:
High or moderate fever
Tiredness and malaise (feeling unwell)
One or more tender red papules or plaques. These enlarge and persist for several weeks. They may have blisters, pustules or ulcers. Sometimes they appear to clear in the centre.
Aching joints and headache
Sore eyes and/or mouth ulcers
Sometimes other organs are affected including bones, nervous sytem, kidneys, intestines, liver, heart, lungs, muscles and spleen.
Skin lesions may arise at the site of injury such as a needle prick, biopsy or insect bite. It some patients they arise only in sun exposed areas.
Sweet's disease is related to pyoderma gangrenosum, and is sometimes difficult to distinguish from it.
In one variant of Sweet's disease, ‘neutrophilic dermatosis of the dorsal hands’, bluish or grey abscess-like nodules arise on the backs of the hands. These nodules may ulcerate. This is sometimes known as ‘pustular vasculitis’ of the hands, because inflamed blood vessels are seen on biopsy as well as an infiltration of neutrophil white cells.
Investigations may reveal:
Raised erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), indicating systemic inflammatory disease
Raised white cell count (neutrophil leukocytosis)
Numerous neutrophil inflammatory cells on skin biopsy associated with broken-up neutrophils (leukocytoclasia) and swelling of cells lining blood vessels (endothelial cells)
Sweets' lesions resolve eventually without leaving a mark or scar, with or without treatment.
Generally there is a single episode of Sweet's disease, but a third of patients may develop recurrent episodes. This is more likely in patients who have underlyng cancer.
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