[Kikuchi disease and systemic lupus erythematosus]

Kikuchi's disease or histiocytic necrotizing lymphadenitis is a rare anatomico-clinical entity affecting young females. It is clinically manifested by generalized lymphadenopathy, fever and neutropenia. Diagnosis depends on histological examination. The etiology is not well known yet, although an autoimmune hypothesis has been suggested. It may be associated with systemic lupus erythematosus. The authors reported a case of Kikuchi's disease associated with apparition of secondary systemic lupus erythematosus. It is about a 16 years old woman who presented with fever, generalized lymphadenopathy, inflammatory polyarthritis and myalgia. Laboratory tests revealed an important inflammatory syndrome with leucopenia. Immunological test were normal and lymph node biopsy was in favour of necrotizing histiocytic lymphadenitis. [Kikuchi disease]. Later on after one year lupus disease was finally identified. Kikuchi's disease is a rare less frequent benign condition, the clinical symptoms resolve spontaneously but a regular Follow up should be taken with these patients to detect secondary onset of another autoimmune disease especially the systemic lupus erythematosus

maladie de gaucher. Traitement enzymatique substitutif a props d'un cas avec revue de la lettirature.

Gaucher's disease, rare, hereditary and potentially mortal affection is characterized by the reduced concentration of the glucocerebroside lipid within the macrophage lysosomes. We report the case of a young 2 years old patient treated by transfusion since he was 9 months because of chronic anemia. According the clinical examination, the general state of the patients was bad with important delayed stanturoponderal growth, a cutaneomucous paller and enormous splenomegaly. The blood count formula showed anemia with major thrombocytopenia. The myelogram was poor and the osteomedullar biopsy showed the presence of Gaucher's cells. The diagnosis has been confirmed by enzymatic dosage [Leucocytar b-glucosidase]. The treatment of the patient has been substitutive enzymatic [inifucerase] with very favorable response. During Gaucher's disease, the enzymatic deficiency results in the pathologic accumulation of the substrate [glucocerebroside] in the lyososomes, this metabolic overloading may cause polyvisceral disease with spontaneous evolution after mortal. The recent discovery of a recombining glucocerebrosidase [imiglucerase] transformed the prognosis of this disease

Inflammatory pseudo-tumour of the liver in child: a case report

The inflammatory pseudo-tumour is a very rare benign tumour in the child. Its hepatic localization leads to diagnosis difficulties particularly with malignant tumours. We report the case of a 17 month-old boy who had an enormous abdominal mass which was 18 cm diameter. The hepatic origin has been visualized by echography and magnetic resonance imaging. The diagnosis of liver inflammatory pseudo-tumour has been realized after surgical biopsy. The treatment consisted in total resection of the tumour, taking the liver left lobe, with good clinical evolution and good recession of 34 months. The diagnosis of the liver inflammatory pseudo-tumour is histologic. It may be based on the needle biopsy of the tumour assisted by the imaging, which allows to have a conservative attitude for these benign tumours