Fulminant mulch pneumonitis in a previously healthy child.

Chronic granulomatous disease (CGD) is associated with multiple and recurrent infections. In patients with CGD, invasive pulmonary infection with aspergillus species remains the greatest cause of mortality. Acute fulminant presentations of fungal pneumonia are catastrophic. It is a medical emergency, and currently the treatment is based on association of corticosteroids and antifungal therapy. We describe the case of an 11-year-old boy, with late initial presentation of CGD, which was revealed by fulminant aspergillus pneumonia. He was successfully treated with an association of high doses of steroids and voriconazole.

[Dermatopolymyositis induced by D-penicillamine in rheumatoid polyarthritis. Apropos of 1 case with review of the literature]. / Dermatopolymyosite induite par la D-pénicillamine au cours de la polyarthrite rhumatoïde. A propos d'un cas avec revue de la littérature.

Treatment of rheumatoid arthritis (RA) with D-penicillamine (DP) is associated with development of dermatopolymyositis (DPM) in 0.2 to 1.2% of cases. A case of DPM which developed after four years DP therapy in a 58-year-old female with RA is reported. The favorable outcome after discontinuation of DP and administration of corticosteroids and the absence of recurrence or malignant disease after 4 years 9 months follow-up demonstrated the causal relationship between DP therapy and development of DPM. An analysis of 34 previously published cases of DP-induced DPM (DP/DPM) showed the following: development of DPM was not influenced by the dosage or duration of DP therapy; reported cases of DP/DPM were clinically identical with primary DPM but had a different outcome, with permanent recovery of DP/DPM occurring 1.5 to 6 months after withdrawal of DP; patients with DP/DPM had immune disorders, including antinuclear antibodies in 14 of 34 patients; the high prevalence of the B18, B35, DR4 haplotype in these patients denotes immunogenetic differences with primary DPM patients (B8-DR3) and DP-induced myasthenia (DR1).

[Gougerot-Sjögren syndrome associated with dysthyroidism]. / Syndrome de Gougerot-Sjögren associé aux dysthyroïdies.

The occurrence of Gougerot-Sjögren syndrome in a context of dysthyroidism is not a chance happening, as is reflected by the 37% rate of incidence. Routine labial biopsy, carried out in 59 cases of primary hypothyroidism due to atrophic thyroiditis, 20 of Basedow's disease and 4 of Hashimoto thyroiditis identified Gougerot-Sjögren syndrome (3-4 Chisholm grade) in 3 of the 59 cases of hypothyroidism i.e. in 5 percent, and in one Basedow case out of 20, i.e. 5 percent. Using Kaplan's criteria, the incidence of Gougerot-Sjögren syndrome rises to 22 percent in hypothyroidism and 30 percent in Basedow's disease. Consequently, the incidence of Gougerot-Sjögren syndrome is higher in Basedow disease than in primary hypothyroidism. Gougerot-Sjögren syndrome is seen particularly in dysthyroidism involving manifest and all-round clinical signs. This association does not seem to result from one disease being caused by the other, but the outcome of the onset of entities to which a genetically determined context predisposes the subject, as is shown by the prevalence of HLA DRW3 and HLA B8 groupings in Gougerot-Sjögren syndrome, in primary hypothyroidism, in Basedow's disease and in Hashimoto's thyroiditis.

[Acute renal insufficiency revealing an acute lymphoblastic leukemia (apropos of a case)]. / Insuffisance rénale aiguë révélatrice d'une leucémie aiguë lymphoblastique (à propos d'un cas).

We report the observation of a 40 year old man who was admitted with acute renal failure. Renal biopsy revealed massive interstitial infiltration by lymphocytes. Bone marrow examination was diagnostic for acute lymphoblastic leukemia. This presentation of acute leukemia is unusual. The mechanism of renal failure is discussed.

[Intrathoracic localization of Behçet's disease]. / Localisations endothoraciques de la maladie de Behçet.

Five case histories of intra-thoracic involvement occurring in the course of Behcet's disease were studied. These included a superior vena caval obstruction, an obstructive syndrome, transitory infiltrates, repeated lung infections and a case of haemoptysis. A review of the literature was carried out "à propos" of each case. The blood results seemed to be the involved as a manifestation of all the varied clinical presentations. The advantage of specialised investigations such as pulmonary angiography, perfusion scintigraphy and bronchial arteriography is underlined.