Sweet's syndrome notes

This should be read after Chapter 3: Sweet's Syndrome or just before, whatever you prefer.

These notes are a summary of the disease (with medical terms) based on scientific articles (the links are further down).

Remember I AM NOT A DOCTOR, I am a biomedical student.

This case is based on other real-life examples.

The first case that Aya works on, all the treatment in the novel is fantasy, here is included some of the modern-life alternatives.

Sweet's syndrome: acute febrile neutrophile dermatosis

SS was first described by Robert Douglas Sweet in 1964.

This condition is a reactive process characterized by the abrupt onset of tender, red-to-purple papules and nodules that coalesce to form plaques. These plaques usually occur on the upper extremities, face, or neck and are typically accompanied by fever and peripheral neutrophilia.

"Sweet syndrome (acute febrile neutrophilic dermatosis) is a hypersensitivity reaction that occurs in response to systemic factors, such as hematologic disease, infection, inflammation, vaccination, or drug exposure. The condition is neutrophil-mediated, as evidenced by its histopathologic appearance, associated neutrophilia, and response to medications that affect neutrophil activity." [1]

There are three types of Sweet Syndrome.

-Classical or idiopathic

-Malignancy-associated (for example to cancer)

-Drug-induced

The most common type is idiopathic, which is predominant in women.

Case: Aidan veim Azura.

Symptoms:

- Pyrexia of 38.7ºC

-Painful erythematous, presence of nodules and plaques on the upper arms, shoulders and neck

-Still recuperating from a bronchitis

- Doesn't have signs of leukocytoclastic vasculitis

Summary of laboratory findings:

-Biopsy of nodules: presence of neutrophilia: 9000cell/uL

- Blood test: Erythrocyte sedimentation rate of 24mm/hr

- hs CPR: 3.1mg/L

- Doesn't have signs of any anemia

- Normal platelet count: 316,000/mcL

Deduction: Sweet's Syndrome

Treatment: For the bronchitis amoxicillin (750mg), for the fever and pain paracetamol (1gr) alternating each 4hr with ibuprofen (650mg) if the fever is still presented high, prednisone 1 mg/kg/day.

Summary:

Diagnostic criteria for classical SS

Note: presence of major criteria (1 and 2) and at least, TWO of the four minor criteria (3, 4, 5 and 6).

[7]

Major criteria

Abrupt onset of tender or painful erythematous plaques or nodules, occasionally with vesicles, pustules, or blisters

Predominantly neutrophilic dermal infiltrate without leukocytoclastic vasculitis

Minor criteria

Preceded by a nonspecific respiratory or gastrointestinal tract infection or vaccination or associated with:

–Inflammatory diseases such as chronic autoimmune disorders, infections

–Hemoproliferative disorders or solid malignant tumours

–Pregnancy

Fever > 38°C

Abnormal laboratory values at presentation (three of four):

–Erythrocyte sedimentation rate > 20 mm/h

–Elevated C‐reactive protein levels

–Leukocytosis > 8,000

–Neutrophilia > 70 %

Excellent response to treatment with systemic corticosteroids or potassium iodide

Links:

[1] https://emedicine.medscape.com/article/1122152-overview#a4

[2] https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-2-34

[3] https://www.sciencedirect.com/science/article/pii/S221300711200024X

[4] https://journals.lww.com/co-hematology/fulltext/2013/01000/Acute_febrile_neutrophilic_dermatosis__Sweet_s.6.aspx

[5] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293911/

[6] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4890080/

[7] https://onlinelibrary.wiley.com/doi/full/10.1111/ddg.13350