M Bucchia1,2, S Barbarot3, H Reumaux4, M Piram5,6, E Mahe7, S Mallet8, X Balguerie9, A Phan10, J-P Lacour11, S Decramer12, Y Hatchuel13, S Jean14, E Begon15, A Joubert3, E Merlin16, D Wallach17, U Meinzer2,18,19, E Bourrat2,20. 1. Centre Hospitalier Le Mans, Service Urgences pédiatriques, Le Mans, France. 2. Centre de référence des rhumatismes inflammatoires et maladies auto-immunes systémiques rares de l'enfant (RAISE), Service de pédiatrie générale, Maladies Infectieuses et Médecine Interne, Hôpital Robert Debré, Paris, France. 3. Service de Dermatologie, CHU de Nantes - Hôtel Dieu, Nantes, France. 4. Service de Pédiatrie et médecine générale, CHRU de Lille, Hôpital Jeanne de Flandre, Lille, France. 5. CHU de Bicêtre, Service de Rhumatologue Pédiatrique, CEREMAIA, Le Kremlin-Bicêtre, France. 6. CESP, U1018 Inserm, Université Paris-Sud, Le Kremlin-Bicêtre, France. 7. Service de Dermatologie, Unité de Soutien à la Recherche Clinique, Argenteuil, France. 8. Service de Dermatologie de l'hôpital de la Timone, Aix-Marseille Université, Marseille, France. 9. Clinique Dermatologique, CHU de Rouen, Rouen, France. 10. Service de Néphro-Rhumato-Dermatologie Pédiatrique, Hospices Civils de Lyon, Hôpital Femme-Mère-Enfant, Bron, France. 11. Service de Dermatologie, Centre Hospitalier Universitaire de Nice, Nice, France. 12. Centre Hospitalier Universitaire de Toulouse, Service de Néphrologie Médecine Interne Pédiatrique, Hôpital des Enfants, Centre De Référence des Maladies Rénales Rares du Sud Ouest, Institut National de la Santé et de la Recherche Médicale (INSERM), U1048, Institut of Cardiovascular and Metabolic Disease, Université Toulouse III Paul-Sabatier, Toulouse, France. 13. Service de Pédiatrie, Centre Hospitalier Universitaire de Martinique, Fort de France, France. 14. Service de pédiatrie Centre Hospitalier universitaire de Rennes, Rennes, France. 15. Service de Médecine, Centre Hospitalier René-Dubois, Cergy Pontoise, France. 16. CHU Clermont-Ferrand, Pédiatrie Générale Multidisciplinaire, CIC INSERM 1405, Clermont-Ferrand, France. 17. Médecin (honoraire) des Hôpitaux de Paris, Paris, France. 18. INSERM UMR1149, Université Paris Diderot, Paris, France. 19. Institut Pasteur, Unité Biologie et génétique de la paroi bactérienne, Paris, France. 20. Service de Dermatologie, Centre Hospitalier Universitaire Saint-Louis, Paris, France.
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.
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.
Authors: Emma H Weiss; Christine J Ko; Thomas H Leung; Robert G Micheletti; Arash Mostaghimi; Sarika M Ramachandran; Misha Rosenbach; Caroline A Nelson Journal: Curr Dermatol Rep Date: 2022-03-16