This is a report of a dermatological illness with symptoms affecting the whole body which should not be ignored when patients present in Primary Care.
She is a 55-year-old woman known to have both high blood pressure and COPD. No allergies were noticed, she smoked 10 cigarettes each day and had been prescribed enalapril for her hypertension for 6 years, while using inhaled formoterol for the last 2 years. Because the pulmonologist observed a worsening trend in the patient’s lung disease, they decided to replace the patient’s formoterol with a combination of indacaterol and glycopyrronium inhaled capsules.
She went to the Primary Care clinic due to pain, erythematous patches and a mild fever on her face and neck after she started her new inhaler treatment two days earlier (Fig. 1). She said she had not used a new cosmetic, kept her usual diet and still had sun exposure, but always with protection. There had been no catarrhal signs at all in the days leading up to her visit.
She was referred to a Dermatologist for an examination of the lesions. At the recommendation of the dermatologists, the patient was told to stop using the medicine and afterward the doctors performed a biopsy as well as tests for a complete blood count, antibodies, lupus anticoagulant and serology. Oral corticosteroids were given to help lower the inflammation.
After 24 to 48 hours, the area of the lesion is less severe and the pain reduces. The blood tests detect increased white blood cells (neutrophilic), presence of antibodies and lupus anticoagulant, together with negative serology tests.
The findings from the skin biopsy were received after twenty days and they confirmed a diagnosis of Sweet syndrome.
Histopathologically, sweet syndrome (also called acute febrile neutrophilic dermatosis) displays neutrophils in the skin and because of this, it falls within neutrophilic dermatoses.
Clinically, it shows up as reddened papules or plaques on both sides of the body, usually not matching perfectly, appearing all at once and causing pain. Many people have tumors most often in the face, neck, upper trunk or hands. In addition, these infections are identified by systemic symptoms such as fever, with many white blood cells (leukocytosis) and most of them neutrophils (neutrophilia)1,3 .
Lists of symptoms to identify Sweet syndrome
Major criteria
Appearing all of a sudden are erythematous or violaceous plaques or nodules that cause pain.
The skin tissue contains neutrophils not as a result of vasculitis.
Minor criteria
Reactions before the actual fever starts
Leukocytosis
When arthralgia, conjunctivitis, fever or a hidden neoplasia (cancer) are present
The way a patient handles systemic corticosteroids
An increased amount of erythrocytes settling in a period of time
Exactly what causes Sweet syndrome is not clearly understood. A few studies suggest that cytokines are responsible for helping neutrophils and histiocytes get activated and respond to infections 4,5 . Usually, an upper respiratory infection or paraneoplastic diseases can trigger a hypersensitivity reaction, in addition to consuming any type of medication 2,6,7 . Knowing what you are dealing with matters, especially ruling out categories of Sweet syndrome such as those on the hands which sometimes appear on their own in cancer or bleeding disorders.
When the cause is drugs or unknown (idiopathic), women are more likely to be affected 2.
Contraceptives, antiepileptics, antibiotics, antihypertensives, colony-stimulating factors and vaccines are the drugs most likely to be associated with Sweet syndrome 6 . Even so, there are other medicines involved in exclusivity disputes which is why this clinical case was introduced.
In most cases, when oral corticosteroids are used as first-line treatment for Sweet’s syndrome, pain and general discomfort disappear in the beginning hours and the lesions go away within a week 2 .
Here, where facial and neck lesions suddenly become erythematous and papular, the differential should include urticaria, contact dermatitis and toxicoderma since that is how they look and also cutaneous lupus, as the lesions share similar properties. After history and all the tests pointed to different diseases and with the certain conclusion from the biopsy, the patient was diagnosed with Sweet syndrome.
Because Sweet’s syndrome can be triggered by different drugs, it is important to consider it as a possible diagnosis when the connection between drug use and the outbreak of lesions is obvious. Reported cases of Sweet’s syndrome caused by inhalant therapy are nonexistent, so this is the first; thus, it is important to factor other medications into consideration, even without direct studies describing them.
Since very few cases are known, this is not among the first diagnoses to check, unless some infection, inflammatory bowel disease, autoimmune connective tissue problems or hematological malignancies are suspected.
For this reason, after making a diagnosis of Sweet syndrome, tests should be done to rule out possible tumors, illnesses from rheumatic diseases or infections.
A good starting point is for primary care doctors to consider all the possible illnesses, regardless of whether a diagnosis is determined in their office. Knowing about rare diseases, a primary care physician makes sure patients are referred early and appropriately for diagnosis and care.
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