Current Concepts in the Etiology and Treatment of Behçet Disease

Recurrent aphthous stomatitis (RAS; aphthae; canker sores) is common worldwide. Characterised by multiple, recurrent, small, round, or ovoid ulcers with circumscribed margins, erythematous haloes, and yellow or grey floors, it usually presents first in childhood or adolescence. Its aetiology and pathogenesis is not entirely clear, but there is genetic predisposition, with strong associations with interleukin genotypes, and sometimes a family history. Diagnosis is on clinical grounds alone, and must be differentiated from other causes of recurrent ulceration, particularly Behcet disease - a systemic disorder in which aphthous-like ulcers are associated with genital ulceration, and eye disease (particularly posterior uveitis). Management remains unsatisfactory, as topical corticosteroids and most other treatments only reduce the severity of the ulceration, but do not stop recurrence.

Oral mucosal disease: recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is one of the most common oral mucosal disorders. Nevertheless, while the clinical characteristics of RAS are well-defined, the precise etiology and pathogenesis of RAS remain unclear. The present article provides a detailed review of the current knowledge of the etiology, pathogenesis, and management of RAS.

Recurrent Aphthous Stomatitis

The etiology and pathogenesis of intracranial aneurysms are clearly multifactorial, with genetic factors playing an increasingly recognized role. Intracranial aneurysms have been associated with numerous heritable connective tissue disorders, which account for at least 5% of cases. Of these disorders, the most important are Ehlers-Danlos syndrome Type IV, Marfan's syndrome, neurofibromatosis Type 1, and autosomal dominant polycystic kidney disease; the association with intracranial aneurysms, however, has been firmly established only for polycystic kidney disease. Familial intracranial aneurysms are not rare but account for 7 to 20% of patients with aneurysmal subarachnoid hemorrhage and are generally not associated with any of the known heritable connective tissue disorders. First-degree relatives of patients with aneurysmal subarachnoid hemorrhage are at an approximately fourfold increased risk of suffering ruptured intracranial aneurysms, compared to the general population. Various possible modes of inheritance have been identified in families with intracranial aneurysms, suggesting genetic heterogeneity. Although the benefits have never been quantified, screening for asymptomatic intracranial aneurysms should be considered in families with two or more affected members. The yield of such a screening program may approximate 10%. Although it is unlikely that there is a single gene with major effect, much effort is currently being directed at locating intracranial aneurysm genes.

Genetics of intracranial aneurysms.

Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature.

Resume Propos Le thalidomide est le traitement de reference des aphtoses orogenitales recurrentes. La posologie habituelle est de 100 mg/j, mais une posologie plus faible serait aussi efficace avec une moindre toxicite. Afin d'evaluer le meilleur ratio efficacite/toxicite, nous avons conduit une etude pilote de 1993 a 1996. Methodes Dix-sept patients presentant une aphtose recurrente ont ete inclus (âge moyen: 43 ans, H/F: 12,5): huit aphtoses buccales recurrentes; trois aphtoses orogenitales recurrentes; quatre maladies de Behcet; deux leucemies myelomonocytaires. La dose initiale de thalidomide etait de 50 mg/j (un comprime) pendant 1 mois. En cas de remission, la posologie etait diminuee a un comprime 1 jour sur 2 le mois suivant, puis un comprime tous les 3 jours le 3emois. Un electromyogramme etait realise initialement et tous les 6 mois. Resultats Dix patients ont eu une remission complete a 1 mois et sept ont ete ameliores. Parmi ceux-ci, six etaient en remission complete apres 2 mois et un apres 4 mois. La remission se prolongera a la posologie de un comprime 1 jour sur 2 chez 12 patients. Dix d'entre eux essaieront un comprime tous les 3 jours et quatre d'entre eux rechuteront. Parmi les six restants qui essaieront un comprime tous les 4 jours, on notera une rechute. La duree moyenne de traitement est de 20,4 mois (2–48). L'electromyogramme montera une diminution des potentiels d'action sensitifs chez six patients, mais seulement trois d'entre eux arreteront le traitement pour cause de parcsthesie dans deux cas et d'areflexie dans un cas. Le delai moyen d'apparition de la neuropathie sera de 8 mois et demi, sans correlation avec la dose quotidienne ingeree. Conclusions Notre etude confirme que 50 mg/j de thalidomide sont efficaces comme posologie initiale et qu'un comprime tous les 2 ou 3 jours est suffisant pour maintenir la remission chez plus de 60 % des patients. Le thalidomide est un traitement sur dans le traitement de l'aphtose recurrente si les criteres de surveillance sont respectes.

Traitement de l'aphtose récurrente par thalidomide à faible dose. Étude pilote chez 17 patients

Resume Propos. – Les myelodysplasies sont des hemopathies clonales developpees a partir d’une cellule souche totipotente. Un desordre immunologique primitif est discute ; cette hypothese pourrait impliquer une association non fortuite avec des maladies systemiques. C’est ce que nous avons voulu verifier dans ce travail. Patients et methodes. – Soixante cas de myelodysplasies diagnostiquees dans le service entre 1990 et 1999 (groupe I) ont ete analyses retrospectivement. La frequence des maladies systemiques dans le groupe I a ete comparee a celle d’un groupe de 120 temoins (groupe II) apparies par l’âge et le sexe, hospitalises dans le meme service pendant la meme periode. Resultats. – L’âge moyen des patients etait de 83 ans. Il y avait 52 % d’anemies refractaires avec exces de blastes, 43 % d’anemies refractaires, 5 % d’anemies sideroblastiques. Quatorze cas de maladies systemiques ont ete decouverts (23 %) contre 4 % dans le groupe temoin (p  Conclusions. – L’association maladies systemiques – myelodysplasies ne semble pas fortuite, supportant ainsi l’hypothese d’un desordre immunitaire commun aux deux types d’affections. En outre, la description de deux cas d’amyloses systemiques AA et celui de pyoderma gangrenosum suggere l’existence d’une anomalie fonctionnelle supplementaire des macrophages et granuleux.

Myélodysplasies et maladies systémiques. Une association non fortuite

Ingrown toenail and indinavir: case-control study demonstrates strong relationship.

Type IV Ehlers-Danlos syndrome, a rare disease caused by abnormal synthesis of type III collagen, often leads to vascular fragility We report 9 cases (6 men and 3 women, mean age 35 years) For 7 of the patients, the inaugural signs were arterial complications including haemoperitoneum in 2 patients with multiple aneurysmal dystrophy of the abdominal arteries, one case of ruptured subclavian artery, two dissections of the renal artery, one case with rupture of a cerebral aneurysm, one rupture of the mesenteric artery and a haematoma after arterial puncture Other vascular manifestations were acrosyndrome (n = 4), varicose veins (n = 3), and prolapsus of the mitral valve (n = 2) In addition, 8 of the 9 patients presented extravascular signs There was a history of familial disease in 5 cases Pregnancy was completed to term in three patients: a cesarean section was required in one case and intra-uterine growth retardation was seen in 2 Morbidity was important with hemiparesia, blindness and paraparesis sequellae One patient died from haemorrhage This series of patients with type IV Ehlers-Danlos syndrome illustrates the severity of this disease whose prevalence is often underestimated The disease is transmitted by autosomal dominant inheritance, underscoring the importance of familial testing for early diagnosis Clinicians should be aware of the vascular manifestations and avoid invasive punctures or operations except in exceptional indications

[Vascular and/or cardiac manifestations of type IV Ehlers-Danlos syndrome. 9 cases].

Alteration in the production of free oxygen radicals and proinflammatory cytokines by peritoneal and alveolar macrophages in old mice and immunomodulatory effect of RU 41740 administration. Part I: Effect of short and repetitive noise stress.

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