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.

[POEMS syndrome]. / La sindrome POEMS.

The POEMS syndrome is a rare multisystem disorder of obscure pathogenesis characterized by polyneuropathy, organomegaly, endocrinopathy of various forms, production of monoclonal component, and skin changes. This syndrome occurs only in about 1% of plasmocytoma cases, but in more than 50% of the rare osteosclerotic subtypes and plasma cell dyscrasias. Therefore it is frequently associated with multicentric Castleman's disease. Chronically elevated serum levels of proinflammatory cytokines associated with a weak or even decreased of anti-inflammatory cytokines, is a feature of this syndrome. Recently, the overproduction of serum vascular endothelial growth factor may be relevant to the pathogenesis of most the manifestations including neuropathy.

[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.

[A particular case of iodine-induced hypothyroidism]. / Un particolare caso di ipotiroidismo iodio-indotto.

A surplus of iodine taken through drugs or food can cause a functional thyroidal deficiency, especially if there are pre-existent alterations in the gland. The case described of a young woman with a iodine-induced hypothyroidism without goitre due to an excessive trans-cutaneous/trans-mucous absorption of iodine in sea-bath salts. The woman, aged 32, showed a symptomatology characterized by asthenia, ponderal increase, constipation and bradypsychia. The thyroid appears normal after echography and scintigraphy. Iodized sea-bath salts have been used for three months in a hot water bath for a slimming effect. A complete regression is noticed with their suspension after one year. An excessive supply of iodine can even if rarely cause a hypothyroidism with/without goitre, because of an intrinsic defect in the mechanism of the organic link which permits a persistent Wolff-Chaickoff effect. A genetic predisposition is supposed and also a higher frequency in patients with a preceding thyroiditis, Basedow disease or Hashimoto disease. Particular attention should be given to a differential diagnosis in case of hypothyroidism in young women with a normal thyroid and negative antibodies. It is clear that a careful pharmacological anamnesis is essential as well as a careful anamnesis on the way of life.

[Adult coeliac disease, epilepsy and cerebral calcifications. A case report]. / Malattia celiaca dell'adulto, epilessia e calcificazioni cerebrali. Descrizione di un caso clinico.

Coeliac disease has a prevalence of between 1/300 and 1/2000 in Europe. A net increase has been observed in the past few years and has been correlated with the greater attention focused on this pathology. The clinical signs may be heterogeneous and their onset may occur at any age. In addition to the classic presentation of the typical malabsorption syndrome, an ''atypical'' presentation has been described, especially in ''adults'', with more frequent involvement of extraintestinal sites and more often in a mono/paucisymptomatic form. The classic variety of coeliac disease is currently thought to be merely the tip of an iceberg whose submerged parts are represented by the subclinical variations, correlated clinical syndromes not yet fully identified and an asymptomatic variety (silent, latent or potential) characterised by various degrees of permanent or transient diet-related alterations to intestinal mucosa. Recently, the association between coeliac disease and epilepsy was described in patients with bilateral occipital calcifications. This study reports the case of a 36-year-old man with manifestations of late onset coeliac disease after a long history of epilepsy starting in infancy and associated with cerebral calcifications.

[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.