Sweet Syndrome With Vasculitis

[Shell567]

Sweet Syndrome With Vasculitis: Time To Adopt a New Criteria? cureus.com/articles/170637#!/

Existing diagnostic criteria excludes vasculitis (inflammation of the blood vessels which is normally absent on biopsy), despite more and more cases of Sweet's syndrome with vasculitis being reported.

Steroids continue to be the mainstay of treatment, and a good response to steroids continues to be a part of the diagnostic criteria, although newer treatments have been used and proved effective.

"We present a case of Idiopathic SS with vasculitis along with a brief review of the existing literature."

CASE-STUDY.

- 79 year old female patient with skin rash on both palms, thighs and knees.

- Over next few days, fevers.

- Skin biopsy showed lots of neutrophils, fluid in the dermal skin layer, and vasculitis. Some of the neutrophils looked like histiocytes (can be seen in the histological variant, histiocytoid SS).

"The diagnostic criteria for classical SS was first proposed by Su and Liu in 1986 . This was subsequently revised by Von Den Driesch."

"The requirement for absence of vasculitis makes the diagnosis questionable in many cases."

"Although response to steroids is a component of the diagnostic criteria, no established guidelines exist for management of SS."

ADDITIONAL NOTES.

Vasculitis is sometimes seen in SS, particularly the variant, neutrophilic dermatosis of the hands. However, this is often not seen as a true vasculitis, but caused by neutrophils releasing noxious substances and severe inflammation.

Sweet's syndrome and histiocytoid SS.

In SS, mature white blood cells called neutrophils accumulate in the tissues. In histiocytoid SS immature myeloperoxidase-positive cells and not mature neutrophils can be seen (mainly M2-like CD 163+macrophages). Myeloperoxidase is an enzyme that’s abundantly expressed in neutrophils.

The immature cells in histiocytoid SS can be mistaken for histiocytes; immune cells that destroy foreign substances and help fight infection. Sometimes, real or authentic histiocytes may also be present.

[sweets2010]

My husband is N of 1 for SS where we live. He sometimes becomes a guinea pig when it comes to treatment. He gets Vasculitis very easily and quite often it turns to Sepsis when an infection gets into his system. He has been in and out of hospital for the last 14 months.

Sometimes, I take the books I have kept on his rollercoaster ride thru the last 13 years with SS. I have taken pictures of the various rashes to show what happens if he is not properly treated and his SS gets out of control. It usually blows them away and then they take me seriously.

I would definitely like to see new Criteria for Vasculitis

[Shell567 Administrator Sweet's Syndrome UK in reply to sweets2010]

Hi Sweets2010,

Sorry to hear your husband has been repeatedly ill. Is the vasculitis part of his SS or did he develop SS secondary to vasculitis?

[sweets2010 in reply to Shell567]

I believe that vasculitis is part of SS. He has very thin skin due prednisone and every time the Doctor tries to lower his prednisone below 13/14 mg he becomes subseptible to getting an infection. If I don't get him to the ER quickly his SS gets activated and it is hard getting under control. He usually ends up with Sepsis. After talking with the hematologist it has been decided that no doctor or specialist will drop his prednisone below 15 mg. It's just too risky. It's been 14 months and he is still struggling with his health.