The following is a clinical case of dermatological disease with systemic repercussions that we must take into account in the Primary Care consultation.
This is a 55-year-old woman with a medical history of hypertension and COPD. She had no allergies, smoked 10 cigarettes daily, and had been on regular treatment with enalapril for 6 years and inhaled formoterol for 2 years. Due to a worsening of her lung disease, the pulmonologist decided to reinforce her inhaled treatment by discontinuing formoterol and prescribing a combination of indacaterol and glycopyrronium in inhaled capsules.
She came to the primary care clinic because on the second day of her new inhaled treatment, she developed painful, erythematous plaques on her cheeks and neck, accompanied by a low-grade fever (Fig. 1). The patient denied using any new cosmetics, reported no changes in her usual diet, and did report sun exposure, although with adequate protection. She also reported no cold-like symptoms in the preceding days.
An urgent referral to Dermatology was decided upon for evaluation of the lesions. After being seen by the Dermatology Service, she was advised to discontinue the new medication, and a biopsy and blood tests were performed, including a complete blood count, antibodies, lupus anticoagulant, and serologies. Oral corticosteroids were prescribed.
Within 24-48 hours, the lesions decrease in intensity and the pain subsides. Blood test results reveal the presence of leukocytosis with neutrophilia, and negative antibodies, lupus anticoagulant, and serologies.
After 20 days, the results of the skin biopsy arrive, and they are positive for Sweet’s syndrome.
Sweet’s syndrome (also known as acute febrile neutrophilic dermatosis) is a dermatological disease classified within neutrophilic dermatoses, since histopathologically it is characterized by the presence of neutrophil infiltrates1.
Clinically, it is characterized by the appearance of erythematous papules or plaques with a bilateral, generally asymmetrical distribution, sudden onset, and pain. The most frequent locations are the face, neck, upper trunk, and hands. It is also characterized by the presence of systemic symptoms (fever, leukocytosis with neutrophilia, etc.)2 (Table 1)1,3.
Table 1.
Diagnostic criteria for Sweet’s syndrome
| Major criteria | Minor criteria |
|---|---|
| Sudden appearance of painful erythematous or violaceous plaques or nodules. Dermal infiltration of neutrophils without vasculitis. | Prodromal symptoms of fever or infectious process ; Leukocytosis; Association of arthralgia, conjunctivitis, fever, or underlying neoplasm; Response to systemic corticosteroids; Increased erythrocyte sedimentation rate |
The etiology of Sweet’s syndrome is not fully understood. According to some studies, the pathogenesis may be mediated by cytokines involved in the chemotaxis and activation of neutrophils and histiocytes.<sup>4,5</sup> Presumably, a hypersensitivity reaction is triggered by a prior process, such as an upper respiratory tract infection or paraneoplastic processes, and also by the use of certain medications.<sup>2,6,7</sup> Diagnosis is important, as it requires ruling out systemic disease, such as atypical forms of Sweet’s syndrome on the dorsum of the hands, which can be the main sign of occult neoplastic diseases.<sup>8</sup>
In cases secondary to drugs or idiopathic, it is more frequent in the female sex2.
The drugs most frequently shown to be correlated with Sweet’s syndrome are contraceptives, antiepileptics, antibiotics, antihypertensives, colony-stimulating factors, and vaccines.6 However, these drugs are not the only ones involved, which is why we have presented this clinical case.
The first-line treatment for Sweet’s syndrome is oral corticosteroids, which respond quickly, so that general discomfort and pain subside within the first few hours and the lesions disappear in less than a week2.
In this case of sudden onset of erythematous papular lesions on the face and neck, the differential diagnosis must include urticaria, contact dermatitis, and toxicoderma due to the way the lesions appeared, and cutaneous lupus due to their characteristics. Once all of these were ruled out through the patient’s history and the results of complementary tests, and thanks to the conclusive diagnosis of the biopsy, the patient was diagnosed with Sweet’s syndrome.
Sweet’s syndrome can be induced by a large number of drugs and should be considered as a diagnostic possibility, especially if the relationship between drug exposure and the appearance of the lesions is clear. To date, no cases of Sweet’s syndrome secondary to inhalation therapy have been described; this is the first. Hence the importance of considering other drugs, even if they have not been described in the literature.
Since it is a rare disease, it is not usually established as a differential diagnosis at first, but it should be taken into account especially because it can involve not only the skin, but also internal organs, being a marker of infections, inflammatory bowel disease, autoimmune connective tissue diseases and malignant neoplasms, especially of hematological origin.
Therefore, it is essential that after the diagnosis of Sweet’s syndrome, the study be completed by ruling out associated organic conditions, such as tumors, rheumatological diseases, or infections.
Primary care physicians must consider all possible diagnoses, even if a definitive diagnosis cannot be made during a consultation. A primary care physician’s knowledge of rare diseases leads to timely and appropriate referrals, enabling patients to be diagnosed and treated promptly.
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