Neonatal Sweet Syndrome: A Potential Marker of Serious Systemic Illness

Abstract

Sweet syndrome is an inflammatory disease characterized by fever and painful erythematous plaques with a dermal neutrophilic infiltrate. It is most common in adults, where it is often parainflammatory or paraneoplastic, but is rare in children. We describe 3 cases of neonatal Sweet syndrome, including 1 patient who had myelodysplastic syndrome and immunodeficiency, the first report of a premalignancy underlying infantile Sweet syndrome. We reviewed the literature on patients presenting with neutrophilic dermatosis in the first 6 months of life. Of 20 cases, 6 had a probable viral etiology, 4 primary immunodeficiencies, 3 neonatal lupus syndrome, 1 gastrointestinal involvement, 1 HIV, and 5 probable genetic cases. Three of these had chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome, caused by mutations in the PSMB8 gene. Most children who presented within the first 6 weeks of life had either a serious underlying condition, such as primary immunodeficiency, or a genetic Sweet syndrome, with 2 fatalities among this latter group. The outcome of postinfective cases was good. Extracutaneous involvement was unusual, whereas postinflammatory scarring and cutis laxa occurred in a minority of patients. In conclusion, Sweet syndrome in the neonatal period often heralds a serious underlying disorder and requires thorough investigation.

Age‐specific characteristics of neutrophilic dermatoses and neutrophilic diseases in children

“…Interestingly, all of them were <1‐year‐old infants (at the age 2, 6 and 7 months). In the literature, neutrophilic diseases involving the upper airways and causing respiratory distress have reported in two additional patients presenting with PG and one with SS . Similar to our observations in this study, ENT involvement (laryngeal lesions) occurred before the age of one year in both cases (at the age of 4 and 9 months) and caused severe respiratory distress.…”

Progressive erythematous papules and plaques in an infant

“…The average age of onset of SS in children is 5 years. Contrary to the female dominance in adults, there is reported to be an equal sex predisposition in children, with a male dominance in infants . A diagnostic criteria for SS was put forth by von den Driesch in 1994 (Table ) .…”

“…Contrary to the female dominance in adults, there is reported to be an equal sex predisposition in children, with a male dominance in infants. [4][5][6] A diagnostic criteria for SS was put forth by von den Driesch in 1994 (Table 1). 1 Our patient had onset of lesions at the age of 13 months, and fulfilled the criteria proposed for SS.…”

“…1 Based on its aetiology, SS has been classified into five categories: (i) classic idiopathic; (ii) parainflammatory (including inflammatory and autoimmune diseases); (iii) paraneoplastic (including malignant and premalignant conditions); (iv) pregnancy-associated; and (v) drug-related. [4][5][6] Paediatric SS most often falls within the classic idiopathic type, which often follows a transient nonspecific infection. Based on two major reviews of SS in children, classic idiopathic, parainflammatory and paraneoplastic SS account for approximately 42%, 33% and 25% of cases respectively.…”

New Pustular Lesions in an Infant with Fever

“…Sweet syndrome in children is rare, accounting for approximately 5% of all cases. Neonatal and infantile Sweet syndrome are even more uncommon; a 2012 review found 12 infants presenting within the first 8 weeks of life . It is characterized by rapid onset of tender erythematous papules or plaques with a predominant neutrophilic infiltrate on histopathology without leukocytoclastic vasculitis.…”