Sweet's syndrome associated with monosomy 7 myelodysplastic syndrome.

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a reactive skin process frequently associated with inflammatory and neoplastic diseases, but particularly with hematologic malignancies. It usually precedes the underlying disorders for months or even years. Much of the evidence for this is based on a small series of case reports and reviews of the literature. Recently, immunological theories have suggested that helper T cell type 1 is involved in the pathogenesis of Sweet's syndrome. This process causes stimulation of the cytokine cascade, which may be responsible for the local and systemic activation of neutrophils and histiocytes. Clinically, Sweet's syndrome is characterized by an acute eruption of painful erythematous or violaceous plaques or nodules with fever, malaise, neutrophilic leukocytosis, and an elevated erythrocyte sedimentation rate. Peripheral blood neutrophilia is frequent and is one of the diagnostic criteria. However, 53% of patients with Sweet's syndrome linked to hematologic malignancies do not present any neutrophilia but rather granulocytopenia. Abnormal functioning of neutrophils is possible in many diseases. We report a case of a middle-aged male patient presenting Sweet's syndrome and granulocytopenia due to myelodysplasia and an anomalous chromosome seven (7-) with poor prognosis.

[Renal involvement in Waldenstroms macroglobulinemia. Case report and review of the literature]. / In process citation.

The author reports a patient with Waldenstroms macroglobulinemia and infiltration of the kidney interstitium with lymphoplasmacytoid cells. In contrast to multiple myeloma, infiltration of the kidney interstitium with lymphoplasmacytoid cells is not a rare event in Waldenstrom s macroglobulinemia at all. Our case reported showed enlargement of the kidney with a diffuse increase in the cortical echogenicity and the presence of hypoechogenic areas scattered throughout the kidney, there was no renal or perirenal masses. The treatment with chlorambucil and prednisone was ineffective, and she has a good response to a polychemotherapy regimen. The role of fine-needle aspiration biopsy performed under ultrasound guidance is emphasized.

[Sweet syndrome: personal experience and review of the literature]. / La sindrome di Sweet. Esperienza personale e revisione della letteratura.

The Sweet's syndrome or acute febrile neutrophilic dermatosis is a well characterized cutaneous disease frequently associated with inflammatory and neoplastic diseases, particularly haematologic malignancies, and usually precedes the underlying disorders for months or years. Much of the evidence for this is based on case reports, small series of cases and reviews of the literature. Recently, immunologic theories suggest that the pathogenesis of Sweet's syndrome is probably mediated through helper T cell type 1 cytokines (IL-2, gammaINF) rather than helper T cell type 2 cytokines (IL-4). This results in the stimulation of a cytokine cascade, which might be responsible for the local and systemic activation of neutrophils and histiocytes. Clinically, Sweet's syndrome is characterized by the acute onset of an eruption of painful erythematous or violaceous plaques or nodules with fever, malaise, neutrophil leucocytosis and a raised erythrocyte sedimentation rate. Personal experience in 4 cases observed during five years is described. All of them presented diagnostic criteria of this disease and had a good response to corticotherapy and/or indomethacin. A review of the literature on this uncommon syndrome is also reported.

[Melkersson-Rosenthal syndrome. Presentation of a clinical case and review of the literature]. / Sindrome di Melkersson-Rosenthal. Presentazione di un caso clinico e revisione della letteratura.

Melkersson-Rosenthal syndrome is a rare form of hereditary angioedema characterised by a triad of symptoms of which incomplete (oligo- or monosymptomatic) forms have been described, frequently associated with dysreactive diseases or neoplasia. The authors describe the case of a 48-year-old man with an incomplete form which was successfully treated with steroids and, after a careful and detailed revision of the literature on the subject, they make a number of etiopathogenetic, histopathological, clinical and therapeutic comments.