atypical presentation of juvenile dermatomyositis: about one case

Juvenile dermatomyositis is a multisystem immune-mediated disease that affects the skin, muscles and gastro-intestinal tract. Elevation of serum muscle enzymes, electromyogram findings and muscle biopsy are helpful to confirm the diagnosis of dermatomyositis. The goal of this article is to describe an unusual presentation of juvenile dermatomyositis, associated to celiac disease, with atypical exploration and unexpected evolution to extensive calcinosis. Nora, 14 years old, is followed since the age of 10 for celiac disease successfully treated with a gluten-free diet. At 11 years she is hospitalized because of arthralgia and myalgia. The physical examination shows erythematous facial rash, Gottron papules in metacarpo-phalangian articulations, and desquamatives lesions of elbows. She presents ankles, knees and wrists arthritis with lumbalgia. The diagnosis of dermatomyositis is suspected. Laboratory findings show a moderate inflammation, non auto-immune signs, increased level of muscular enzymes, without any abnormalities neither in the electromyogram nor in the biopsy. The diagnosis of dermatomyositis is confirmed. The patient is treated with high doses of oral corticotherapy, articular kinesi-therapy and gluten-free diet. The outcome was satisfactory, but an extensive calcinosis appeared after 3 years. Dermatomyositis and celiac disease is a rare association; only a few cases have been reported. They have common auto-immune condition that can explain this association. Regarding the nonspecific results of the electromyogram and the muscular biopsy, it can be due to the choice of an area which is not involved. The calcinosis is unusual in this type of monocyclic and stabilized involvement. It can appear due to the poor efficacy of oral corticoids prescribed in the context of malabsorption

Hypertension portale chez l'enfant. [a propos de 75 cas]

This study had based on 75 cases of the portal hypertension [PHT] at the children's hospital of Casablanca at the Pediatry 3, during a period of 12 years [January 85 to December 97]. The age of our patients varies from 4 months to 16 years. The patients had presented a splenomegaly in 69 cases [92%], a digestive haemorrhage in 42 cases [56%], a collateral venous circulation in 46 cases [68%], an ascites in 40 cases [53%] and a hepatomegaly in 20 cases [27%]. In 58 fibroscopy examinations, the esophageal varices were found in 52 cases [89%]. The echography objectived PHT's signs in all the cases. The etiology was the cirrhosis [37 cases]: post-hepatitis [16 cases], metabolic [12 cases] and biliary [6 cases]. The portal venous obstruction was observed in 15 cases. The authors wanted to emphasize the management's difficulties of variceal bleeding in our context