Article

Extracutaneous Sweet Syndrome Involving the Gastrointestinal Tract in a Patient With Fanconi Anemia

Division of Pathology and Laboratory Medicine, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
Journal of Pediatric Hematology/Oncology (Impact Factor: 0.96). 02/2001; 23(1):59-62. DOI: 10.1097/00043426-200101000-00015
Source: PubMed

ABSTRACT Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a cutaneous eruption characterized clinically by the appearance of painful red plaques and nodules and histologically by an intense dermal neutrophilic infiltrate. Extracutaneous manifestations are rare. We report a patient in whom otherwise typical cutaneous Sweet syndrome was accompanied by an extracutaneous manifestation in the ileum.

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    • "Fanconi anaemia (FA) is a genetic disease characterized by chromosomal fragility and an increased risk of haematopoietic disorders including MDS and AML (Alter, 2003). SS in the setting of FA has been reported in five cases in the literature (Baron et al, 1989; McDermott et al, 2001; Chatham-Stephens et al, 2008). We describe seven patients with FA and SS and report a more frequent association than is seen in other premalignant conditions. "
    British Journal of Haematology 04/2011; 154(2):278-81. DOI:10.1111/j.1365-2141.2011.08604.x · 4.96 Impact Factor
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    ABSTRACT: The objective of this study was to describe the clinical features of Sweet syndrome in children. Our study population consisted of seven children diagnosed with Sweet syndrome over a 22-year period. Age, sex, appearance and location of lesions, associated signs and symptoms, past medical history, pathology, and subsequent disease course were documented for each patient. Fever and typical lesions were reported in most of patients in our study. The majority of patients presented with less-typical findings, such as pustules, vesicles, bullae, oral ulcerations, atrophic scars, and evidence of pathergy. Of the seven children in our study, four were found to have a preceding nonspecific upper respiratory or gastrointestinal infection, and two were diagnosed with an underlying hematologic malignancy. Our results suggest that atypical lesions are relatively common in children with Sweet syndrome and that underlying malignancy is associated with a minority of cases of pediatric Sweet syndrome.
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