Neonatal Sweet syndrome: a potential marker of serious systemic illness.

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.

Congenital refractory anaemia with vacuolisation of bone marrow precursors, sideroblastosis and growth failure in a girl with normal endocrine pancreatic function.

The case is described of an 8-years-old girl with consanguineous parents. She was apparently well, apart, from growth retardation, until 18 months of age when she developed severe normocytic hypochromic anaemia. Bone marrow examination revealed vacuolisation of the erythroid and myeloid precursor, and electron microscopic studies showed striking sideroblastosis with ringed arrangement of the iron granules. Porphyrin metabolism was apparently normal, whereas blood levels of iron and ferritin were high. A careful study of the exocrine pancreas showed completely normal function. Vitamin B6 administration was unsuccessful. The patient is transfusion-dependent, and iron chelation treatment has produced good results. The case could be a new entity or a variant of congenital sideroblastic anaemia, since it has some features in common with the syndrome described by Pearson et al.