Association of Sweet’s Syndrome and Be... - Sweet's Syndrome UK

Sweet's Syndrome UK

488 members298 posts

Association of Sweet’s Syndrome and Behçet’s Disease

Shell567 profile image
Shell567AdministratorSweet's Syndrome UK
4 Replies

Association of Sweet’s Syndrome and Behçet’s Disease: Is It Possible? A Case Report, Sep 2023. pubmed.ncbi.nlm.nih.gov/377...

See comments for info on Behcet's and Sweet's syndrome. Both are forms of neutrophilic dermatosis (white blood cells called neutrophils migrate to, infiltrate and accumulate in the skin). Behcet's causes blood vessel inflammation throughout the body (systemic vasculitis). Inflammation of the blood vessels can sometimes be seen in Sweet's, but this isn't a true vasculitis (caused by neutrophils releasing noxious substances and severe inflammation).

"We report the case of a 43-year-old patient followed for Behçet’s disease, who developed cutaneous plaques of neutrophilic dermatosis of both upper limbs. The clinical and biological picture was in favor of Sweet’s syndrome."

- 43-year-old woman followed since 2005 for Behçet’s disease

- Pseudofolliculitis on the thighs and back (skin condition associated with Behcet's)

- Treated with colchicine 1 mg/day with a good clinical outcome (a few episodes of mouth ulcers, but complete resolution of skin lesions, including genital lesions)

- For one year, the patient stopped colchicine without medical advice. Developed skin lesions on both forearms and hands with outbreaks of mouth ulcers. Also, fever and fatigue.

On clinical examination:

- Temperature of 38.7 °C

- Circular skin lesions

- Two recent mouth ulcers and genital lesions

- Erythema nodosum lesions on both legs (skin condition associated with Behcet's)

- Pathergy test positive (see comments).

Skin biopsy from forearm showed Sweet's syndrome. Can develop secondary to Behcet's. Treated with steroid medication.

"Digestive fibroscopy showed pangastritis and histology revealed the presence of Helicobacter pylori." Patient treated for Helicobacter pylori-mediated gastritis (stomach infection caused by H. pylori bacteria).

"Studies have shown that the two diseases are genetically distinct. Mizoguchi observed an elevated frequency of HLA B51 and HLA-DQw3 in patients with Behçet’s disease, while HLA-Bw54 was significantly higher in patients with Sweet’s syndrome."

Both Behcet's and Sweet's "involve a cascade of cytokine activation leading to an influx of neutrophils and other immune cells."

What is a cytokine cascade?

A cytokine cascade occurs when your immune system releases too many molecules or proteins called cytokines. These molecules promote inflammation and can overstimulate the activity of other immune cells.

"The occurrence of Sweet’s syndrome during a flare-up of Behçet’s disease suggests a very probable pathogenic link between these two entities..."

Written by
Shell567 profile image
Shell567
Administrator
To view profiles and participate in discussions please or .
Read more about...
4 Replies
Shell567 profile image
Shell567AdministratorSweet's Syndrome UK

Sweet's syndrome dermnetnz.org/topics/acute-...

Behcet's dermnetnz.org/topics/behcet...

Shell567 profile image
Shell567AdministratorSweet's Syndrome UK in reply toShell567

Pathergy dermnetnz.org/topics/pathergy

Alex97 profile image
Alex97 in reply toShell567

Hi, I know this post is a few months old but I have bern diagnosed with Behcets for about 12 years and approx 5 years ago diagnosed with sweets syndrome. Interesting reading, thank you

Shell567 profile image
Shell567AdministratorSweet's Syndrome UK

Thanks Alex97!

Not what you're looking for?

You may also like...

What are the symptoms of Sweet’s syndrome? Does it only affect the skin?

The main symptom of Sweet's syndrome (acute febrile neutrophilic dermatosis) is skin lesions that...
Shell567 profile image
Administrator

An Introduction to Sweet's syndrome - links to information (pinned post)

Sweet’s syndrome is an autoinflammatory (not autoimmune) condition and form of neutrophilic...
Shell567 profile image
Administrator

Does anyone have problems with pathergy?

Pathergy is when lesions can develop in an area where the skin is damaged or irritated. It affects...
Shell567 profile image
Administrator

[CASE-STUDY] Infantile histiocytoid Sweet syndrome without an underlying systemic association, OCT 2021

"Pediatric Sweet syndrome is rare, accounting for an estimated 5% to 8% of all Sweet syndrome...
Shell567 profile image
Administrator

Mouth ulcers and Sweet's syndrome

I know that some members of this group have problems with Sweet's syndrome-associated mouth ulcers,...
Shell567 profile image
Administrator

Moderation team

Shell567 profile image
Shell567Administrator

Content on HealthUnlocked does not replace the relationship between you and doctors or other healthcare professionals nor the advice you receive from them.

Never delay seeking advice or dialling emergency services because of something that you have read on HealthUnlocked.