Search

Resume La maladie de Still de l'adulte pose dans la majorite des cas des problemes therapeutiques, compte tenu des effets indesirables des traitements necessaires. C'est pourquoi nous avons teste en ouvert l'action des immunoglobulines intraveineuses, une therapeutique a faible risque iatrogene qui a ete reconnue efficace dans d'autres maladies multisystemiques. Sept malades atteints de maladie de Still de l'adulte ont recu une a huit cures d'immunoglobulines par voie intraveineuse a la dose de 1g/kg/j 2 jours de suite. Une reponse definie par le passage en remission clinique a ete obtenue chez les sept patients. Elle a dure de 1 a 90 jours chez trois patients qui ont ensuite rechute; elle se poursuit actuellement depuis en moyenne 13 mois (2 a 24 mois) chez les quatre autres. Aucun parametre clinique ne permettait de caracteriser a posteriori les bons repondeurs aux immunoglobulines intraveineuses. Ces donnees, qui doivent etre confirmees par une etude controlee, laissent esperer un progres therapeutique pour la moitie des patients atteints de maladie de Still de l'adulte qui repondent bien aux immunoglobulines intraveineuses.

We report here a case of chronic inflammatory bowel disease revealed by multiple large cutaneous aseptic distal necrotic ulcers.A 44-year-old male presented with high fever at 40°C associated with multiple necrotic abcesses located on the distal part of his limbs. They were treated successfully by debridment and dressings associated with antibiotics allowing complete healing after 1 month. Six months later, the patient relapsed on his left hand with a short episode of diarrhoea. A total coloscopy revealed a Crohn's disease. Systemic corticotherapy and azathioprine were administered and complete remission was obtained with a 1-year follow-up.Skin manifestations that this patient presented were atypical because of their number, size, and location, exclusively distally on the limbs. This report illustrates an unusual presentation of Crohn's disease with multiple necrotic ulcers only located on the patient extremities.

We review the current knowledge about fibromyalgia, adding to the clinical aspects, the nosology, epidemiology and pathogenesis. The therapeutic and social management of these suffering patients are discussed.The limitations of the American College of Rheumatology classification criteria used as diagnostic criteria are discussed. Fibromyalgia is not a simple psychiatric disorder, even if psychiatric symptoms are constantly found. Based on functional brain imaging, there is some evidence pointing to an abnormal function of the supra-spinal centres for pain regulation.Fibromyalgia is a clinical autonomous entity. Physiopathology knowledge is improving, but must be confirmed by new research. Patients will take profit of multimodal individualized treatment programs, including explanations about the diagnosis. In most cases, fibromyalgia is compatible with the maintenance of a professional activity, possibly adapted to the patient. Recognized disability requiring compensation is infrequent.

In most cases, the treatment of adult's Still disease presents difficulties, in view of its undesirable side-effects. For this reason, we made an open trial of the effects of high-dose intravenous immunoglobulins (IVIg), a therapy with low iatrogen risk, and whose effectiveness in the treatment of other multisystemic diseases has been acknowledged. Seven patients suffering from adult Still's disease were given between one and eight IVIg infusions with a dose of 1 g/kg/day for two consecutive days. All seven responded positively, with clinical improvement. It lasted between 1 and 90 days in three patients, who subsequently relapsed; it has been continuing for an average of 13 months (2 to 24 months) in the other four patients. There were no clinical features making it possible to distinguish, after the trial, the patients who responded positively to intravenous immunoglobulins. This data, which needs to be confirmed with a controlled trial, provides hope of improved therapy for the one half of patients suffering from adult Still's disease who respond positively to intravenous immunoglobulins.

Four cases of association of hyperparathyroidism and vasculitis are presented. This association seems to be a forfuitus event, although in 2 cases, onset of both diseases was concomitant.