Age‐specific characteristics of Neutrophilic Dermatoses and Neutrophilic Diseases in Children
M. Bucchia S. Barbarot H. Reumaux M. Piram E. Mahe S. Mallet X. Balguerie A. Phan J.‐P. Lacour S. Decramer Y. Hatchuel S. Jean E. Begon A. Joubert E. Merlin D. Wallach … See all authors
First published: 05 June 2019 https://doi.org/10.1111/jdv.15730
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/jdv.15730
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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 pediatric 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 pediatric 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 was 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 pediatric 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 pediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes.
M. Bucchia S. Barbarot H. Reumaux M. Piram E. Mahe S. Mallet X. Balguerie A. Phan J.‐P. Lacour S. Decramer Y. Hatchuel S. Jean E. Begon A. Joubert E. Merlin D. Wallach … See all authors
First published: 05 June 2019 https://doi.org/10.1111/jdv.15730
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/jdv.15730
ePDFPDFTOOLS SHARE
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 pediatric 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 pediatric 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 was 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 pediatric 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 pediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes.
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