[Systemic mastocytosis: a case with diffuse bone involvement]. / Mastocitosi sistemica: un caso con diffuso coinvolgimento osseo.

The authors report the case of a 54-year-old woman with systemic mastocytosis with cutaneous, gastroenteric and skeletal involvement. The patient, who had presented wine-coloured skin lesions for years diagnosed as urticaria pigmentosa on the basis of the skin biopsy, was admitted to the Institute of Internal Medicine owing to the radiological findings of severe osteopenia. Instrumental and laboratory tests led to the correct diagnosis of type 1 systemic mastocytosis. The peculiarity of the case in question prompts the authors to recall the classification of mastocytosis, namely those pathological syndromes characterised by tissular infiltration by mast-cells, and to outline elements regarding its differential diagnosis in relation to disorders such as various forms of carcinoids and osteopenia, and in particular osteoporosis and osteolysis secondary to metastatic processes.

Benign recurrent intrahepatic cholestasis. Some reflections on a case followed for 20 years.

Benign recurrent intrahepatic cholestasis (BRIC) is a form of cholestasis of obscure aetiology characterized by recurrent episodes of jaundice and itching associated with a morphological picture of pure intrahepatic cholestasis. No effective treatment has yet been found among the many that have been proposed and the invariably benign nature of the condition has been questioned. A case of BRIC followed for a period of 20 years is described. This case is of great interest from these two points of view: 1) the histologic and electron microscopic findings 23 and 41 years after the first episode of cholestasis, respectively, failed to reveal evidence of the possible future development of cirrhosis; 2) treatment with ursodeoxycholic acid proved ineffective both therapeutically and in the prevention of episodes of bile stasis: on the contrary, calculosis of the common bile duct appeared after 8 months from the onset of the treatment.

[Angioimmunoblastic lymphadenopathy with dysproteinemia]. / La linfoadenopatia angioimmunoblastica con disprotidemia.

Angioimmunoblastic lymphadenopathy with dysproteinemia. Angioimmunoblastic lymphadenopathy with dysproteinemia is a lymphomatous-like disease associated with typical anatomopathological features of the lymph nodes and severe dysproteinemia. The clinical course is variable. Acquired immunodeficiency, oral infections, neoplastic development are frequently present. The evolution of the disease is also variable; spontaneous resolution as well as lethal complications are possible. No specific therapy is available. There are conflicting opinions about the nosographic statement of angioimmunoblastic lymphadenopathy among benign lymphadenopathy and lymphomas.

[Angioimmunoblastic lymphadenopathy with dysproteinemia. Personal case reports]. / La linfoadenopatia angioimmunoblastica con disprotidemia. Osservazioni personali.

Angioimmunoblastic lymphadenopathy with dysproteinemia. Case report. We report nine cases of angioimmunoblastic lymphadenopathy with dysproteinemia. Initial symptoms were not specific in any case nor was a specific drug involved as a possible cause. In every case we observed a diffuse enlargement of the lymph nodes and severe dysproteinemia (with hyper or hypo-gammaglobulinemia). Death during the first year was observed for four patients.

[Diphosphonates. Current trends and therapeutic prospects]. / I difosfonati. Attualità e prospettive terapeutiche.

Diphosphonates are compounds characterized by a P-C-P bond. They are thus analogs of pyrophosphate and can be useful for treating several bone diseases. The authors synthetically review the mechanism of action of these drugs and their most important clinical applications. The authors finally mention the interesting therapeutic possibilities deriving from the development of new members of this class.

[Fibrodysplasia ossificans progressiva or Münchmeyer's disease]. / La fibrodisplasia ossificante progressiva o malattia di Münchmeyer.

Fibrodysplasia ossificans progressiva is a heritable and generalized disorder of connective tissue, characterized by the appearance of bony tissue within the striated muscles, tendons and ligaments; moreover, some skeletal abnormalities may also occur, mainly microdactyly of the big toes. This is a very rare disease, which presents in early life; its course is unavoidably progressive, producing a sort of petrifaction of the patient some years after the first symptoms. It is defined as an autosomal dominant trait, being due in most cases to a new mutation. At present no known treatment is available to stop the course of the disease, but sometimes disodium etidronate may be effective in preventing calcification of heterotopic bony tissue.

[Fibrodysplasia ossificans progressiva or Münchmeyer's disease. Personal observation]. / La fibrodisplasia ossificante progressiva o malattia di Münchmeyer. Osservazione personale.

We report here a case of fibrodysplasia ossificans progressiva in a 14-year-old boy, affected from birth by microdactyly of the big toes. This skeletal abnormality also existed in his paternal great-grandfather. When he was 7, some ectopic ossifications occurred and inexorably progressed despite all therapies. Fibrodysplasia ossificans progressiva is a serious and rare disease with a terrible development, leading to a sort of petrifaction of the patient. The lack of knowledge on this ectopic ossification process explains the want of suitable treatments to stop the course of this disease.

[Hypersensitivity angiitis or microscopic polyarteritis nodosa. Recent findings. III. Pathologic anatomy, etiopathogenetic aspects and therapeutic approach]. / Angioite da ipersensibilità o poliarterite nodosa microscopica. Recenti acquisizioni. Nota III. Anatomia patologica, aspetti eziopatogenetici e approccio terapeutico.

Histologically hypersensitivity angiitis produces necrotising inflammation of the small arterial and venous blood vessels. In most cases the inflammatory infiltrate presents leucocytoclasia i.e. nuclear leucocytic detritus. Unlike polyarteritis nodosa, hypersensitivity angiitis does not affect the medium sized arteries though its lesions are produced at the same stage of development. At skin level, the postcapillary venules are the vessels most often affected. Fibrinoid necrosis of the glomerular loops of the kidney may arise and is often accompanied by epithelial crescents. Aetiologically, a variety of agents--bacteria, viruses, drugs, toxic substances--have been held responsible for the disease, though very often the cause cannot be identified. The most widely based on the finding of immunocomplexes, though other immunological disorders might be involved. Treatment involves the elimination of the antigen held responsible, the suppression of the immune response, the removal of circulating immunocomplexes and the use of anti-inflammatory drugs.

[Hypersensitivity angiitis or microscopic polyarteritis nodosa. Recent findings. II. Clinical aspects]. / Angioite da ipersensibilità o poliarterite nodosa microscopica. Recenti acquisizioni. Nota II. Clinica.

Hypersensitivity angiitis is one of the commonest necrotising vasculitides. Given the ubiquitous distribution of the small blood vessels almost any part of the body may be affected. However benign forms predominantly involving the skin are the most common. Forms predominantly involving the viscera and that may prove fatal are rare. Among the polymorphous skin lesions encountered "palpable purpura" is the most common and its palpability is a vital element in differential diagnosis. Among non-cutaneous sites the kidneys are the most frequent and glomerular involvement is the commonest cause of death. Though laboratory tests provide no specific data they may indicate the severity of visceral involvement.

[Angiitis caused by hypersensitivity or microscopic polyarteritis nodosa. Recent findings. I. Nosographic aspects]. / Angioite da ipersensibilità o poliarterite nodosa microscopica. Recenti acquisizioni. Nota I. Aspetti nosografici.

Hypersensitivity angiitis or microscopic polyarteritis nodosa is one of the necrotising angiitis and was not distinguished from classic polyarteritis nodosa until the Fifties. The present study examines the nosographic aspects of necrotising angiitides with reference to the anatomohistological, aetiopathogenic and clinical criteria proposed by various Authors for their identification and with emphasis on the fact that all these factors must be borne in mind for the purpose of diagnosis. The factors that make it possible to distinguish hypersensitivity angiitis from other necrotising angiitides are, at clinical level, the presence of skin lesions, and in anatomohistological terms the involvement of the small blood vessels and leucocytoclasia.

[Blood hypereosinophilias. IV. Symptomatic hypereosinophilias: connective tissue diseases, neoplasms, blood diseases, various causes]. / Le ipereosinofilie del sangue. Nota IV. Ipereosinofilie sintomatiche: malattie del connettivo, neoplasie, affezioni emopatiche, cause varie.

Among the symptomatic hypereosinophilias and apart from pathologies covered in Note III, diseases of the connective tissue, neoplasias, blood diseases and other conditions are also examined. Two connective tissue diseases often accompanied by hypereosinophilias are Churg and Strauss angiitis and eosinophilic fascitis. Churg and Strauss angiitis (of which 2 personal cases are reported) is a systemic vasculitis usually seen in combination with bronchial asthma and haematic eosinophilia. Eosinophilic fascitis is quite rare and poorly understood. Its symptoms include hardening of the skin and eosinophilia and it is difficult to differentiate from progressive systemic sclerosis. The possible reasons why hypereosinophilia sometimes accompanies benign and more often malignant tumours are discussed. The pathogenesis of the hypereosinophilias encountered in diseases of the blood is still controversial. One hypothesis is that hypereosinophilia is an intrinsic symptom of the blood disease, others believe it to be an immunological response. In this context two personal cases are reported as examples: one of hypereosinophilia in a malignant non-Hodgkins lymphoma, the second in an IgG plasmacytoma. Particular attention was paid to the hypereosinophilia that accompanies the rare blood disease known as angioimmunoblastic lymphoadenopathy with dysproteinaemia (LAID) of which a personal case is reported.

[Blood hypereosinophilias. III. Symptomatic hypereosinophilias: allergic, cutaneous, parasitic, infective, pulmonary and gastro- intestinal diseases]. / Le ipereosinofilie del sangue. Nota III. Ipereosinofilie sintomatiche: malattie allergiche, cutanee, parassitarie, infettive, polmonari, gastroenteriche.

The most frequently observed of the symptomatic hypereosinophilias are those caused by allergic, cutaneous, parasitic, infectious, pulmonary and gastroenteric conditions. Among the allergic conditions, particular attention is paid to the hypereosinophilias caused by allergic asthma, gastroenteritis and reactions to drugs. The most common skin conditions linked to hypereosinophilias such as bullous dermatites and angio-oedema are considered. Turning to the parasitic conditions, the various types of parasite that may produce hypereosinophilias by infesting the organs are examined. The aetiology of tropical eosinophilias and the pathogenetic mechanism that may trigger hypereosinophilias are discussed. It has been thought advisable to group the lung pathologies associated with hypereosinophilias under a separate heading, despite the indubitable importance of the allergic element in these events. Among gastroenteric conditions, the one considered is eosinophilic gastroenteritis whose clinical, anatomopathological and aetiopathogenic features are still not quite clear. Examples of certain forms of secondary hypereosinophilias are given in the form of four unusual personal cases of bronchial asthma, filariasis, an exceptional infestation by Hypoderma bovis and eosinophilic gastroenteritis.

[Hypereosinophilias of the blood. I. Eosinophilic granulocytes]. / Le ipereosinofilie del sangue. Nota I. Il granulocita eosinofilo.

A knowledge of eosinophil granulocytes is indispensable for the study of hypereosinophilia. For this reason, the most recent findings relating to eosinophil morphology, production/regulation mechanism, and function are reported. Particular attention is given to enzyme populations, local control mechanisms and eosinophil cell surface receptors. Among the various enzymes present in the eosinophil, major basic protein (MBP), with its capacity to damage the cells of many organs, plays an important part; other enzymes include eosinophil peroxidase (EPO), arylsulphatase B, phospholipase D, histaminase and cationic proteins (ECP). Factors influencing eosinophil tissue concentrations and mode of action are considered. Recent findings agree on the role of eosinophils in immunological reactions and parasitic infestations: eosinophil plays a part in an immunological physiopathological sequence: it may, act as a killer cell with selective action against invading parasites, or it may be an immune modulator, anti-inflammatory cell able to surround inflammatory reactions and prevent them from spreading.

[Hypereosinophilia of the blood. II. Classification. Benign essential hypereosinophilia. Eosinophilia caused by systemic eosinophilic hemopathies]. / Le ipereosinofilie del sangue. Nota II. Nosografia. Ipereosinofilie benigne essenziali. Ipereosinofilie da emopatia sistemica eosinofila.

Following a previous note on the latest development in the study of eosinophilic granulocytes, a nosographic classification scheme is proposed for the problematic group of haematic eosinophilias. The scheme is based on the division of hypereosinophilias into three basic groups: benign idiopathic hypereosinophilias, hypereosinophiliasis caused by systemic eosinophilic blood diseases and symptomatic hypereosinophilias. Two rare events, hereditary familial eosinophilia and bening, non-familial idiopathic eosinophilia may be added to the benign idiopathic hypereosinophilias group. The group of hypereosinophilias caused by systemic eosinophilic blood diseases is still controversial and difficult to interpret. Particular attention is paid to the so-called "idiopathic hypereosinophilic syndrome" (HES), an umbrella term under which there is a current tendency to group a heterogeneous series of disorders characterised by long-lasting hypereosinophilia where there is no known reason for the increased eosinophilic granulocyte rate. Clinical and physiopathological features are then described to decide whether a given condition lies within the scope of this still little known syndrome.