1/7. Infantile periarteritis nodosa or mucocutaneous lymph node syndrome. A report on four cases and diagnostic considerations.Coronary artery aneurysm in childhood is a rare disease and has in most cases been ascribed to infantile periarteritis nodosa (IPN). In recent years a mucocutaneous lymph node syndrom (MLNS) has been found almost exclusively in japan first described by Kawasaki 1967; this disease frequently involves the coronary arteries and myocardium. Four cases with coronary aneurysms are presented from sweden and seem to be first described from scandinavia. Three of these patients died a sudden death with cardiac arrest. Since MLNS and IPN have identical clinical and pathological features, we suggest that MLNS and IPN constitute a pathologic entity and that to separate them on a clinical or histological basis is nonsensical. The risk of coronary aneurysm and possible sudden death must be considered in patients with uncharacteristic symptoms including prolonged fever, conjunctivitis, exanthema, lesions in the oral mucosa, elevated sedimintation rate, and leukocytosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. An unusual presentation of classic polyarteritis nodosa in a child.Classic polyarteritis nodosa (c-PAN) is a rare disease in adults and extremely rare in children. We report a 3-year-old girl with c-PAN who presented with disturbances of consciousness and hypertension. Cranial tomography showed a subarachnoid hemorrhage. Subsequent magnetic resonance imaging and magnetic resonance angiography demonstrated subarachnoid hemorrhage and acute ischemic lesions. Renal angiography revealed bilateral multiple aneurysms. Due to her constitutional symptoms and hypertension and radiological findings she was diagnosed as having c-PAN. She was successfully treated with hydralazine followed by angiotensin-converting enzyme inhibitor, calcium channel blocker, intravenous pulse methylprednisolone, and subsequently oral prednisolone and oral cyclophosphamide. To our knowledge this is the youngest patient with c-PAN presenting with subarachnoid hemorrhage. Malign hypertension at this young age deserves a meticulous investigation of the vascular origin. Furthermore, treatment with pulse methyl prednisolone followed by oral prednisolone and oral cyclophosphamide is a successful modality of treatment in such a life-threatening presentation of c-PAN in childhood.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Painful rash and swelling of the limbs after recurrent infections in a teenager: polyarteritis nodosa.polyarteritis nodosa is a rare disease in childhood and adolescence that is difficult to diagnose clinically. We report on a 17-y-old girl presenting with a history of recurrent infections of the upper respiratory tract and conjunctivitis followed by a painful rash on the upper and lower extremities resembling erythema nodosum. The diagnosis of polyarteritis nodosa was proven by skin biopsy. Therapy with intravenous immunoglobulins failed, but with systemic steroids she responded promptly. CONCLUSION: polyarteritis nodosa is a differential diagnosis in adolescents presenting with fever and an erythema nodosum-like rash.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. polyarteritis nodosa of the epididymis in a patient with Whipple's disease.The case of a 55-year-old white male who developed necrotizing arteritis localized to the superior pole of the epididymis is presented. He had a history of Whipple's disease and euthyroid Graves' disease. Histopathologic section of an extratesticular mass showed a necrotizing vasculitis with giant cells; periodic acid Schiff stain for Whipple "bacilli" was negative. The combination of rare diseases in our patient suggests the possibility of a common infectious or immune etiology, perhaps mediated via circulating immune complexes.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. polyarteritis nodosa, hairy cell leukemia and splenosis.Appendiceal vasculitis and splenosis in a young women provided pathologic evidence that a systemic necrotizing vasculitis preceded the onset of hairy cell leukemia by at least six years. As in other patients who have had both diseases, florid polyarteritis nodosa developed during the course of the malignancy. However, the antecedent vasculitis, as well as the coincidence of these rare diseases, indicate that systemic vasculitis does not necessarily result from hairy cell leukemia as previously reported and suggest that the two diseases might share a common pathogenesis or predisposing factor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Idiopathic CD4 lymphocytopenia and systemic vasculitis.The syndrome defined as "idiopathic CD4 lymphocytopenia' (ICL) is a rare disease of unknown aetiology, often associated with severe depression of immune defences and the occurrence of opportunistic infections. A case is reported wherein a severe immunodeficiency syndrome with persistent idiopathic CD4 lymphopenia developed in a woman suffering from systemic microscopic polyarteritis; no signs of hiv 1/2 or HTLV I/II infection were evident. The patient died of widespread opportunistic infections. The association of ICL with vasculitis has never been reported until now. A link between the two diseases cannot be ruled out.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Diagnostic value of renal arteriography in polyarteritis nodosa.polyarteritis nodosa (PAN) is a rare disease in childhood. No single pattern of clinical presentation characterizes this disease, but abdominal pain, central or peripheral nervous system disease, arthritis, myalgia and skin lesions occur at some time during the course of the illness. In this case a 16-year-old boy who presented with abdominal pain, elevated sedimentation rate associated with hypertension, and a high level of renin, all of which were detected during his hospitalization, suggested the diagnosis of PAN, and renal angiography was performed. Characteristic renal aneurysms were visualized and the diagnosis was confirmed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |