Scleroderma-like disorders.

Fibrosing disorders comprise a wide spectrum of heterogeneous diseases characterized by sclerosis of the dermis, subcutis, and sometimes the underlying soft tissues and bone. The hallmark of this group of diseases is skin thickening as in systemic sclerosis with a different distribution pattern and for this reason they have also been referred to as "scleroderma-like" disorders. These diseases may have a different clinical course ranging from a benign disease with a localized cutaneous involvement, to a widespread, systemic, life-threatening disease. Some of them are associated with autoantibodies and/or autoimmune conditions. An accurate recognition of these scleroderma-like diseases is important for the institution of the most appropriate treatment.

[Thrombocytopenia associated with brucellosis: a case report]. / Trombocitopenia in corso di brucellosi: descrizione di un caso.

Brucellosis, a common disease in some areas of the world, beside its typical signs and symptoms, as fever, arthropathy, hepatomegaly and splenomegaly, sometimes can complicate into thrombocytopenia, even in severe forms. The pathogenesis of thrombocytopenia in course of brucellosis is variable, and a main role is played by immunological reactions. Authors describe a case report of an eight years child who presented a severe thrombocytopenia in course of acute brucellosis. The patient responded efficaciously to the antibiotic therapy combined with immunoglobulin intravenous therapy.

[Recurrent new-onset uveitis in a patient with rheumatoid arthritis during anti-TNFalpha treatment]. / Uveite recidivante di nuova insorgenza in paziente con artrite reumatoide in terapia con anti-TNFalpha

Inflammation involving the uveal tract of the eye, termed uveitis, is frequently associated with various rheumatic disease, including seronegative spondylarthropathies, juvenile rheumatoid arthritis, Crohn's disease and Behçet's disease. Scleritis and keratitis may be associated with rheumatoid arthritis and systemic vasculitides such as Wegener's granulomatosis. Immune-mediated uveitis can have a chronic relapsing course and produce numerous possible complications, many of which can result in permanent vision loss. Treatment typically includes topical or systemic corticosteroids with cycloplegic-mydriatic drugs and/or noncorticosteroid immunosuppressants, but often there is an insufficient clinical effectiveness. Anti-TNFalpha therapy is promising in the treatment of sight threatening uveitis, particularly in patients with Behçet's disease. However, there have been also reports of new-onset uveitis during treatment of joint disease with TNFalpha inhibitors. We describe a case of new-onset uveitis in a patient with rheumatoid arthritis during therapy with etanercept at first and infliximab at last. Although we cannot exclude uveitis as linked to rheumatoid arthritis, it is unlike that the uveitis arises when the joint disease is well controlled. The hypothetical paradoxical effect of anti-TNF is here discussed.

[Buschke Scleredema, case report]. / Scleredema di Buschke, contributo casistico.

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology, characterized by thickening of the dermis whose characteristics may mainly to mime systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Scleredema can be classified into three clinical groups; each has a different history, course, and prognosis. Each one of these share reduction in chest articular movements and limitation of limbs movements. The skin histology is characterised by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histologic peculiarity. No therapy has been found effective. The authors describe a case of Buschke Scleredema successfully treated by steroids and colchicine. Clinical evaluation of skin induration and thickness as well as ultrasonography were performed at baseline and after treatment.

[Renal abscess in childhood: a case report]. / Ascesso renalein età pediatrica:descrizione di un caso.

We describe the case of a previously healthy six-year-old boy with a right renal abscess due to a methicillin-resistant Staphylococcus aureus, which necessitated a radical nephrectomy. Although renal ultrasonography is often the initial tool to identify fluid collection, in our case the diagnosis was obtained only after performing an abdominal CT with intravenous contrast. It is therefore necessary to combine these different imaging techniques in all children with fever and abdominal/lumbar pain without any apparent cause in order to obtain an early diagnosis and minimise organ damage.