Xanthomatized Sweet’s syndrome: a histological variant

Sometimes, Sweet’s syndrome can occur in an unusual form. This is called a disease variant.

What is xanthomatized Sweet’s syndrome?

Xanthomatized Sweet’s syndrome is a rare histological variant of Sweet’s syndrome.

How many people have xanthomatized Sweet’s syndrome?

In 2016, the first case of xanthomatized Sweet’s syndrome was reported in a 58-year-old man with a history of histiocytoid Sweet’s syndrome and myelodysplastic syndromes (Ferris et al, 2016). It initially appeared as skin lesions on the corners of his mouth.

How is xanthomatized Sweet’s syndrome different from Sweet’s syndrome?

In patients with xanthomatized Sweet’s syndrome, their biopsy results show something different from what you would normally expect to find in Sweet’s syndrome.

In Sweet’s syndrome, lots of mature neutrophils (a type of white blood cell) are found in the tissues. This is often referred to as ‘neutrophilic infiltrate’. In xanthomatized Sweet’s syndrome, both neutrophilic and xanthomatous infiltrate are present.

What is xanthomatous infiltrate?

Xanthomatous infiltrate is a gathering together of foamy histiocytes in the skin. These are histiocytes (immune cells that destroy foreign substances and help fight infection) that are laden with lipids or fats, mainly a fat called cholesterol. A xanthomatous infiltrate is normally found in those with hyperlipidaemia or high levels of fat in the blood, but is occasionally associated with other conditions.

What are the symptoms of xanthomatized Sweet’s syndrome?

The symptoms of xanthomatized Sweet’s syndrome and Sweet’s syndrome are the same. Read more here. 

How is it treated?

Xanthomatized Sweet’s syndrome is treated in the same way as Sweet’s syndrome. Read more here.

References.

Ferris, G., Fabbro, S., Gru, A. and Kaffenberger, J. (2016) Xanthomatized Neutrophilic Dermatosis in a Patient With Myelodysplastic Syndrome. The American Journal of Dermatopathology, Oct 18 (PubMed).

Other.

Sweet’s Syndrome Variants.

© 2012-2017 Sweet’s Syndrome UK

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