European Journal of Pediatrics

, Volume 168, Issue 1, pp 1–9

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases

  • T. Hospach
  • P. von den Driesch
  • G. E. Dannecker
Review

DOI: 10.1007/s00431-008-0812-0

Cite this article as:
Hospach, T., von den Driesch, P. & Dannecker, G.E. Eur J Pediatr (2009) 168: 1. doi:10.1007/s00431-008-0812-0

Abstract

Objectives

The objectives of this study were to analyse the literature on Sweet’s syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet’s syndrome are reported.

Methods

A literature search was performed on Pub med using search terms “sweet* syndrome*” and neutrophil* dermatos*. Patients were subdivided into the following groups: classic/idiopathic, paraneoplastic, and parainflammatory Sweet’s syndrome.

Results

The literature search revealed 64 patients (including our two patients) who were diagnosed with Sweet’s syndrome in childhood and adolescence; 27 (42%) patients were categorized as “classic/idiopathic Sweet’s syndrome”. In 37 patients (58%) chronic associated diseases were reported. Out of these, 21 (33%) patients were categorized as “parainflammatory Sweet’s syndrome” including chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, recurrent infections due to immunodeficiencies, arthritis, and systemic lupus erythematosus. Sixteen (25%) patients were categorized as “paraneoplastic Sweet’s syndrome” comprising both malignant and premalignant diseases like leukemia, aplastic anaemia, and Fanconi anaemia. As all five (8%) patients treated with drugs (granulocyte-colony stimulating factor, retinoid acid) suffered from malignant, premalignant, or parainflammatory diseases, these patients were categorized according to the underlying disease. Two new children with Sweet’s syndrome and associated diseases are presented here, one of them suffering from recurrent infections and trisomy 21, while the other was diagnosed with CNS vasculitis 5 1/2 years after the primary diagnosis.

Conclusions

Sweet’s syndrome should be considered in differential diagnosis of prolonged fever with cutaneous involvement. As most cases of pediatric Sweet’s syndrome are associated with other diseases we suggest careful screening and monitoring of these patients especially concerning malignant/premalignant diseases, immunodeficiencies, cardiovascular involvement, autoimmune diseases, and drug associations.

Keywords

Sweet’s syndromeFebrile neutrophilic dermatosis

Abbreviations

AA

Aplastic anemia

AML

Acute myelogenous leukemia

ALL

Acute lymphoblastic leukemia

ATRA

All-trans retinoic acid

BMT

Bone marrow transplantation

CDA

Congenital dyserythropoeitic anemia

CGD

Chronic Granulomatous disease

CRMO

Chronic recurrent multifocal osteomyelitis

FA

Fanconi anemia

G-CSF

Granulocyte colony stimulating factor

HIV

Human immunodeficiency virus infection

jcML

Juvenile chronic myelogenous leukemia

MBL

Medulloblastoma

MDS

Myelodysplastic syndrome

NBL

Neuroblastoma

SIRS

Systemic inflammatory response syndrome

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • T. Hospach
    • 1
  • P. von den Driesch
    • 2
  • G. E. Dannecker
    • 1
  1. 1.Department of Pediatrics and Pediatric RheumatologyKlinikum Stuttgart, OlgahospitalStuttgartGermany
  2. 2.Department of DermatologyKlinikum StuttgartStuttgartGermany