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

Abstract

Background Our suggested ‘modern’ concepts of ‘neutrophilic dermatoses’ (ND) and ‘neutrophilic disease’ were based on observations in adult patients and have not been studied in paediatric patients. Only a minority of ND occurs in children, and little is known about age‐specific characteristics. Objectives To describe age‐specific characteristics of ND in children and to study whether our suggested ‘modern’ classification of ND may be applied to children. Methods We conducted a retrospective multicentre study in a French cohort of 27 paediatric patients diagnosed with pyoderma gangrenosum (PG) or Sweet's syndrome (SS). Results Demographics and distribution of typical/atypical forms were similar in patients diagnosed with PG and SS. Atypical ND were more frequent in infants (90%), when compared to young children (60%) and adolescents (33%). Neutrophilic disease was observed in 17/27 patients and was most frequent in infants. Neutrophilic disease of the upper respiratory tract, as well as cardiac neutrophilic disease, was only observed in infants, whereas other locations were similarly found in infants, young children and adolescents. In infants and young children, ND were associated with a large spectrum of general diseases, whereas in adolescents associations were limited to inflammatory bowel disease and Behçet's disease. Conclusions Our study describes the concept of ND in paediatric patients and shows that they have some characteristics different from ND occurring in adults. ND occurring in infants can be associated with a large spectrum of general diseases. Occurrence of neutrophilic disease is frequent in children. Thus, ND occurring in young paediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes.

New Practical Aspects of Sweet Syndrome

“…The lesions were distributed primarily on the lower (8/10 patients) and upper extremities (7/10 patients). The investigators noted that nine of the ten patients had extracutaneous involvement and that six of the ten patients had an atypical variant of SS [21]. Thus, it is possible that pediatric SS manifests differently than adult SS, although further studies are needed to more definitively establish the presentation of pediatric SS.…”

Oral tacrolimus for ocular involvement in pediatric neutrophilic dermatoses

“…Four additional patients were described in the French cohort, but information on their underlying ND diagnosis and treatment was not included. 2 Of those patients with ND with ocular involvement, one quarter were less than 2 years old, highlighting that extracutaneous manifestations, including ocular ones, occur more frequently in infants than previously reported. Their presentations included scleritis, episcleritis, palpebral edema, conjunctivis, and superficial keratitis.…”

“…One French case series found ocular involvement in less than 15% of the 27 pediatric patients with PG or SS. 2 Ocular manifestations include conjunctivitis, iritis, scleritis, and retinal vasculitis. 3 Periorbital inflammation and eyelid lesions are also considered ocular manifestations, as eyelid lesions can lead to decreased visual acuity and occasionally vision loss.…”

‘Progressive erythematous papules and plaques in an infant: Sweet that is not so sweet!’: reply from authors

“…He cites a recent article on a French cohort that included 27 children with ND with 17 cases of pyoderma gangrenosum and 10 of SS. 5 The study highlighted a higher risk of extracutaneous manifestations, including respiratory and cardiovascular, and association with autoinflammatory syndrome in children with ND. 5 The limitation of the study was that it is a retrospective study based mainly on the questionnaire and number of SS cases is too small to draw a definite inference.…”

“…5 The study highlighted a higher risk of extracutaneous manifestations, including respiratory and cardiovascular, and association with autoinflammatory syndrome in children with ND. 5 The limitation of the study was that it is a retrospective study based mainly on the questionnaire and number of SS cases is too small to draw a definite inference. Prospectively designed, multicentre, long-term studies including large numbers of patients are required to have a fair idea about prevalence of these associations.…”