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..."
