Mucosal ulcerations revealing primitive hypereosinophilic syndrome.

We report the case of a 27 year-old man developing recurrent oral aphtosis associated with fever and 8 kg of weight loss. Moderate splenomegaly was observed on physical examination and neurological and cardiac examination were normal. Laboratory findings included marked eosinophilia at 3280 giga/l. Bone marrow (BM) examination revealed a myeloproliferative syndrome with mature eosinophils. Splenectomy was performed because of a suspected nodule on the BM, the histopathology revealed a myeloid metaplasia. The diagnosis of myeloproliferative form of hypereosinophilic syndrome (HES) was made. He was treated with interferon-alfa and hydroxyurea. After two years of treatment he had no ulcer recurrence and eosinophil count was at 180 giga/l. Mucosal manifestations as a prodromal symptom of HES are rare. The histology of the lesions shows numerous eosinophils; immunohistochemical analysis confirms the presence of eosinophil peroxydase, major basic protein and eosinophil derived neurotoxin. A few cases have been described. Death occurs 11 months to 5 years after the diagnosis of oral ulcerations. The treatment consists of interferon-alfa and hydroxyurea.

[Inaugural symptoms of Horton's disease in a series of 260 patients]. / Symptômes inauguraux de la maladie de Horton sur une série de 260 patients.

PURPOSE: Horton disease or 'giant cell arteritis' is a known entity in its typical form; the difficulty in diagnosis is due to the atypical signs and symptoms. METHODS: We review 260 medical files presenting Horton disease between 1979 and 1999 in five different departments: three internal medicine departments, one rheumatology department and one geriatric department. RESULTS: The study shows a female domination with a mean age of 75 years. Temporal artery biopsy was done on all patients. Ten patients presented a vascular manifestation. The neurological manifestation was the first symptom in four patients. Five patients had cutaneous symptomatology, with positive temporal artery in three cases. Renal manifestation was present in two patients. Two symptoms are important to discuss because of their frequency: the cough and the peripheral arthritis. We found nine observations with arthritis affecting large joints and responding to nonsteroidal antiinflammatories with positive temporal artery biopsy in seven patients, and 21 observations manifesting by cough without radiological signs; in 57% of cases the temporal artery biopsy was positive, and the cough regressed with corticoids. CONCLUSION: These atypical symptoms have to be known to make a diagnosis and to begin a corticotherapy as soon as possible.

[Clinical or biological symptoms leading to the search for amyloidosis]. / Symptômes cliniques ou biologiques devant amener à rechercher une amylose.

INTRODUCTION: The extracellular, multifocal, disseminated or diffuse localization of amyloidosis accounts for the diversity of clinical presentations and late diagnosis. CURRENT KNOWLEDGE AND KEY POINTS: However, early diagnosis is important, as new drugs have been recently introduced. Both clinical picture and biology help guide diagnosis, including either kidney, heart, skin or neurologic involvement associated with monoclonal gammapathy (primary idiopathic amyloidosis AL); underlying inflammatory or infectious disease, familial Mediterranean fever with proteinuria (secondary amyloidosis AA); cardiac, neurologic or ocular involvement (heredofamilial amyloidosis); carpal tunnel syndrome, joint pain (amyloidosis of hemodialysis). Furthermore, amyloid fibrils are identified on salivary gland biopsy. FUTURE PROSPECTS AND PROJECTS: Due to the introduction of new specific drugs aimed at curing various amyloidoses, early diagnosis is important. Chemotherapy and hematopoietic stem cell transplantation are promising regarding AL amyloidosis, while liver transplantation has proven remarkably successful in heredofamilial amyloidosis. Progress in molecular biology should allow identification of various forms of familial amyloidosis.