Here is a case study of a skin condition that also affects the whole body, so it should receive attention in your Primary Care session.
She is 55 years old and has a history of high blood pressure and COPD. She did not suffer from allergies, usually smoked 10 cigarettes a day and had been treated with enalapril for 6 years and inhaled formoterol for 2 years. Since her lung disease got worse, the pulmonologist decided to discontinue the formoterol treatment and use a combination of indacaterol and glycopyrronium inhaled capsules instead.
She went to the clinic because, soon after starting the inhaler, she noticed red and painful spots on her cheeks and neck, along with a low-grade fever (Fig. 1). She claimed she wasn’t trying any new skin products, didn’t change her regular meal routine and still spent time in the sun with sunscreen. In the days preceding, she had not shown any catarrhal symptoms.
It was planned to refer the patient to the Dermatology section for evaluation of the lesions. Because of the Dermatology Department’s conclusions, the patient was asked to discontinue the medication they were taking and have their blood checked for a biopsy and various tests such as a complete blood count, antibodies, lupus anticoagulant and checks for serology. The doctor gave me oral corticosteroids for the treatment.
In the following 24 to 48 hours, the pain decreases and the redness of the skin reduces. Leukocytosis with neutrophilia is seen together with antibodies, lupus anticoagulant and negative findings for serology on the patient’s blood tests.
After twenty days, the biopsy results returned and showed Sweet syndrome was present.
Sweet syndrome (also referred to as acute febrile neutrophilic dermatosis) is a skin disease that belongs to neutrophilic dermatoses, as it is found in histopathology that various neutrophil infiltrates are present 1 .
Doctors label it by observing bilateral, asymmetrical, erythematous papules or plaques having a sudden and painful beginning. Most melanomas first show up on the face, neck, upper trunk and hands. Systemic symptoms (e.g., fever, an increase in white blood cells with an increase in neutrophils) are often present in such conditions 3 .
The ways experts distinguish Sweet syndrome
Major criteria
Swift onset of painful lesions with a red or purple color.
Presence of neutrophils in the skin without inflammation of blood vessels.
Minor criteria
Early signs of a fever or an infection
Leukocytosis
When linking arthralgia, conjunctivitis, fever or underlying neoplasia, the term ‘Fevered arthritis-conjunctivitis syndrome’ is used.
Reaction to treatment with systemic corticosteroids
Slower blood flow in general
The exact reason Sweet syndrome occurs is not clear. Studies suggest that cytokines cause chemotaxis and the activation of neutrophils and histiocytes to be involved in the disease development 4,5 . Usually, a hypersensitivity reaction begins with an infection of the upper airways, certain disorders linked to cancer cells or after taking specific medicines 2,6,7 . It is necessary to diagnose correctly, because atypical Sweet syndrome on the back of the hand occasionally appears as the main indication of hidden cancer .
Drugs or idiopathic causes usually lead to more cases of acne in women 2 .
Frequently, Sweet syndrome is correlated with medication such as contraceptives, antiepileptics, antibiotics, antihypertensives, colony-stimulating factors and vaccines 6 . Still, some other drugs are also protected by exclusivity and that is the reason we wrote this clinical case.
Both the pain and discomfort of Sweet’s syndrome often improve quickly after a patient starts oral corticosteroids 2.
In such cases where you find erythematous papular lesions on the face and neck, a differential diagnosis is required with urticaria, contact dermatitis, toxicoderma and cutaneous lupus based on how they look and on their characteristics. Once the other conditions were excluded with the patient’s history and complementary tests and the biopsy was done, the patient was conclusively diagnosed with Sweet syndrome.
If a person with Sweet’s syndrome was exposed to medication and the connection between the two is obvious, doctors should consider it as a possible diagnosis. No cases of Sweet’s syndrome following inhalant therapy have been found so far which is why it is essential to keep other potential drugs in mind even when they are not listed in the literature.
As the disease is not common, it might not be thought of at the start, but it should be considered since it can involve the skin and other organs and shows association with infections, IBD, autoimmune diseases and cancers of the blood.
Because of this, a full study is needed after Sweet syndrome is diagnosed to make sure there are no tumors, rheumatic diseases or infections present.
Even if there is not enough information to define the diagnosis, doctors at the primary care level should keep exploring all options. Knowing about rare diseases, a primary care doctor ensures patients get properly referred and treated on time.
The moral obligation to protect people and animals.
The authors do not report any studies involving the participation of humans or animals for this research.
Data confidentiality
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Patients and/or subjects included in the paper have given their informed consent to participate in the study. This document is held by the corresponding author.
