Sweet’s syndrome (SS) is a relatively rare condition in pediatric patients and has a good prognosis, but recurrences do occur and second-line treatment may be required, researchers reported in the International Journal of Dermatology (May 2015; 54(5):518–522).
“In adults, the diagnosis of SS is worrisome because the first concern that comes to mind when treating adults with SS is an associated cancer or leukemia. The association of SS in [our pediatric population of patients] is more related to an infection or [no associated cause]—malignancy is rare,” said Dr. Maria Teresa Garcia-Romero, dermatologist and researcher at Instituto Nacional de Pediatría in Mexico City.
“I always like it when we find this kind of difference between adult patients and pediatric patients because it makes us remember that children are not little adults. Pediatric patients are an entirely different population and their diseases are usually quite different in their manifestation,” said Dr. Garcia-Romero, the lead author of this retrospective study. She was working on a fellowship in pediatric dermatology at The Hospital for Sick Children in Toronto at the time of this investigation.
Characteristics of SS lesions
“The SS lesions have a characteristic erythematous quality. They are papulesand nodules, lesions that give the impression that they may be filled with liquid. The lesions also have a plump, red, and sometimes purplish appearance,” Dr. Garcia-Romero told THE CHRONICLE OF SKIN & ALLERGY.
She added that the SS lesions are really dramatic and SS should be ruled out when these types of lesions are seen by practitioners.
Pediatric patients and SS
First described by Dr. Robert Douglas Sweet in 1964, there have been fewer than 80 pediatric cases of SS reported in the medical literature (Pediatr Dermatol 2012; 29:38–44).
During the analysis period Dr. Garcia-Romero and her colleague Dr. Nhung Ho, a staff dermatologist at The Hospital for Sick Children, retrospectively reviewed the charts of pediatric patients diagnosed with SS both clinically and histologically at The Hospital for Sick Children in Toronto between 2000 and 2012. The authors noted that clinical, laboratory, and pathologic data were analysed, along with patient characteristics and associated diseases, treatment, and long-term outcomes.
According to the authors, they found five patients. Four were male, aged between nine and 14 years who had been diagnosed with SS at the hospital. All of the patients had fever, elevated markers of systemic inflammation, and typical skin lesions.
Data from this series showed that SS was associated with underlying hematologic malignancy in one
patient; all-trans retinoic acid in another; infection in two patients; and in one patient, no identifiable cause was found.
“Most children who have SS according to what we have found are not sick. These pediatric SS patients have systemic symptoms and they sometimes have a fever. Also, neutrophilia and high inflammatory markers are often present.”
Tx of pediatric SS
According to the charts of the five patients from The Hospital for Sick Children in Toronto, three of the five patients treated with systemic corticosteroids had excellent response, and two had recurrences and
received additional treatment with dapsone and saturated solution of potassium iodide.
“The first step in the treatment process is determining if there is an association or something underlining the SS, because after all the SS is a reaction to something,” she said. “If a disease
is found then you should treat the disease first. You should also see if the drugs your patient is on might be associated with SS.”
“In the case of one patient in our series of five patients, she was on a chemotherapy drug called ATRA (all trans retinoic acid) and SS appeared to be associated with that. So if a drug appears to be causing the SS and you stop it then that should be enough to make the SS [resolve], but if that is not the case then you can treat the SS using high doses of steroids—prednisone usually.”
Dr. Garcia-Romero added that another treatment that can be used to treat SS is dapsone. Dapsone is also used in dermatology to treat conditions caused by inflammation, she said.
“So you can try dapsone as a treatment for SS and you can also try a saturated solution of potassium iodine, which can be compounded in a pharmacy, and that works really well also,” said Dr. Garcia-Romero.
- This article was originally published inThe Chronicle of Skin & Allergy (Dec. 2015; 21(8)).