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: 0.9). 02/2001; 23(1):59-62. DOI: 10.1097/00043426-200101000-00015
Source: PubMed


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 · 4.71 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.
    Pediatric Dermatology 01/1981; 29(1):38-44. DOI:10.1111/j.1525-1470.2011.01534.x · 1.02 Impact Factor
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    ABSTRACT: Fanconi anemia (FA) is an autosomal recessive disease associated with an abnormal response to DNA damage. Although FA is well known for the association of aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A review of all reported cases is informative with regard to the specific types of cancer, the ages at which they occur, and the cumulative probability of their development. Medline and bibliographies of publications were searched for articles containing "Fanconi's anemia" or "aplastic anemia" and all cases of FA from 1927 through 2001 were included in the database. Cancer cases were identified within these reports. Descriptive statistical analyses were performed using Stata7 software. One thousand three hundred cases of FA were identified. Nine percent had leukemia (primarily acute myeloid leukemia), 7% had myelodysplastic syndrome, 5% had solid tumors, and 3% had liver tumors. Patients with cancer were older than the cancer-free patients at the time of diagnosis of FA. The median age for cancer (including leukemia) was 16, compared with 68 in the general population. The most frequent solid tumors were aerodigestive and gynecological carcinomas. In approximately 25% of patients with cancer, the malignancy preceded the diagnosis of FA. If the competing risks of aplastic anemia and leukemia could be removed, the estimated cumulative probability of development of a solid tumor in FA patients is 76% by the age of 45 years. Carcinogenic pathways and cancer prevention, surveillance, and treatment can be studied to advantage in this genetic model of human cancer.
    Cancer 01/2003; 97(2):425-40. DOI:10.1002/cncr.11046 · 4.89 Impact Factor
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