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 syndrome (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.

How is it treated?

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

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).

See comments for info. on histological variant, cryptococcoid Sweet’s syndrome.

2012-present, Sweet’s Syndrome UK

5 thoughts on “Xanthomatized Sweet’s syndrome: a histological variant”

  1. New Practical Aspects of Sweet Syndrome, Feb, 2022. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8853033/#CR74

    “While neutrophil predominance is the histopathologic hallmark of SS, cases of SS with a predominant eosinophilic and lymphocytic infiltrate have been described. In cases of eosinophil predominant SS , a complete blood count may be indicated to rule out eosinophilic dermatosis of hematologic malignancy. The authors’ position is that cases without neutrophil predominance should not be classified as SS.

    Recently, a novel subtype of SS, normolipemic xanthomized Sweet syndrome, was reported by Kamimura et al. Microscopic examination of this unusual case revealed CD163-positive foam cells to accompany dense neutrophilic infiltrate extending into the deep dermis. Further reports may aid in further characterization of this variant.”

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  2. ANOTHER HISTOLOGICAL VARIANT: CRYPTOCOCCOID SWEET’S SYNDROME.

    New Practical Aspects of Sweet Syndrome, Feb, 2022. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8853033/#CR74

    “The first reports of cryptococcoid SS are credited to Ko et al., who described this rare histologic variant in 2013. The term ‘cryptococcoid Sweet syndrome’ itself was formally introduced in 2017 by Wilson et al. Microscopic examination of cryptococcoid SS reveals vacuolated mononuclear cells with the presence of basophilic yeast-like bodies, a presentation compatible with a diagnosis of cryptococcosis. However, periodic acid Schiff (PAS) stain of the biopsy fails to reveal fungal elements, and the cutaneous lesions of cryptococcoid SS do not remit with anti-fungal therapy. Cryptococcoid SS appears to be a rare phenomenon, as only four cases of cryptococcoid SS have recently been reported. Of note, all have been associated with ANCA positivity, although only one was present in the setting of primary vasculitis. It is unclear if the association between ANCA positivity and cryptococcoid SS is coincidental or causal.”

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  3. Iodine toxicity after iodinated contrast: New observations in iododerma, 2020. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7109373/

    Iododerma is a rare halogenoderma – a rare skin reaction related to high levels of halogen in the body – that develops after exposure to iodine-containing compounds. This study discusses 3 cases of iododerma in patients who had recent exposure to intravenous iodinated contrast media.

    Case 1: Skin biopsy demonstrated a neutrophilic dermatosis with haloed cryptococcal-like structures. A neutrophilic dermatosis such as bowel-associated dermatosis arthritis syndrome or Sweet’s syndrome with haloed inflammatory cells was considered.
    Case 2: Two skin biopsies demonstrated a dermal neutrophilic infiltrate with cryptococcal-like acellular bodies. Vasculitis was present in 1 of the biopsies.
    Case 3: Skin biopsy showed a dermal neutrophilic infiltrate with haloed structures mimicking Cryptococcus and vasculitis of small and medium-sized vessels.

    Cryptococcoid neutrophilic dermatosis with or without vasculitis should be recognized as part of the histologic spectrum of iododerma.
    ADDITIONAL NOTES: [CASE-STUDY] A hydralazine-induced triumvirate: Lupus, cutaneous vasculitis, and cryptococcoid Sweet syndrome. 2019. Provides an explanation of a cryptococcoid neutrophilic dermatosis – cryptococcoid Sweet’s syndrome. https://www.jaadcasereports.org/article/S2352-5126(19)30506-5/fulltext

    HYDRALAZINE is a medication that lowers blood pressure. It’s associated with both DRUG-INDUCED LUPUS ERYTHEMATOSUS and VASCULITIS (inflammation of the blood vessels). This is an unusual case of hydralazine-induced autoimmune syndrome with elements of both drug-induced lupus and vasculitis, as well as SWEET’S SYNDROME-LIKE skin lesions with distinctive CRYPTOCOCCOID inflammatory cells.

    A woman in her 70s with a history of high blood pressure, type 2 diabetes mellitus, bladder cancer and chronic KIDNEY DISEASE developed JOINT PAIN, eye swelling, weeping from eyes and blisters over her extremities and trunk. Medications included hydralazine, amlodipine, clonidine, aspirin, clopidogrel, furosemide, simvastatin, and insulin. Eye swab showed no eye infection, but the antiviral, valacyclovir, was started as a precaution. Blood tests showed raised erythrocyte sedimentation rate and C-reactive protein, indicating increased levels of inflammation in the body. There were concerns that these results along with blisters might be part of vasculitis.

    Steroids (prednisone 60 mg daily) were started, but after 4 days of treatment, eye and skin symptoms still hadn’t improved. Physical examination showed severe eyelid ectropion (lower eyelid turns or sags outward, away from your eye), SWOLLEN SKIN PAPULES on the face, mouth lesions, and larger blister-like areas on the arms and legs. There was also some swelling of both knee joints. Blood tests showed raised white cell count, ANAEMIA (normocytic), and raised creatinine attributed to obstruction of the ureter. Culture results were negative, as well as specific tests for mycoplasma, herpes simplex, varicella zoster, Aspergillus, Cryptococcus, and histoplasma.

    Skin biopsy of the skin lesions showed a dense NEUTROPHILIC INFILTRATE (lots of white blood cells called neutrophils in tissues); prominent FIBRINOID NECROSIS (cell death of vessel walls); neutrophilic degeneration; haemorrhage. There were also small-appearing, unusual cells surrounded by vacuolated SPACES resembling the polysaccharide capsule of the yeast-like fungus, CRYPTOCOCCUS NEOFORMANS. However, tests showed that the cells were myeloid in nature, e.g. neutrophils. Serologic analyses showed positive antinuclear antibodies (ANA); anti-histone antibodies; anti-double stranded DNA antibodies (anti-dsDNA – a group of ANA); positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) and anti-Myeloperoxidase antibodies (anti-MPO).

    The patient was started on high-dose intravenous steroids (solumedrol 500 mg daily) with flattening of the skin lesions and decrease in eyelid ectropion. She was then discharged on an oral steroid taper (prednisone 60 mg daily).

    DISCUSSION.
    Hydralazine-induced lupus is more commonly reported and less severe than hydralazine-induced vasculitis, which can cause severe lung and heart problems. This study shows that overlap can exist between these 2 conditions. A history of joint pain and normocytic anaemia, as well as positive ANA, anti-dsDNA, and anti-histone antibodies, are consistent with drug-induced lupus. Skin (dermal) vessel necrosis with positive p-ANCA and anti-MPO antibodies point toward drug-induced vasculitis. The patient also had features of Sweet’s syndrome – swollen skin lesions and neutrophilic infiltrate on biopsy – but vasculitis is rare in this condition. The vacuolated spaces on biopsy are thought to represent ballooning degeneration of neutrophilis that can mimic Cryptococcus in both vasculitis and Sweet’s syndrome (CRYPTOCOCCOID SWEET’S).

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