[Dermatological features of auto-inflammatory recurrent fevers]. / Peau et fièvres récurrentes auto-inflammatoires.

Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations.

[Diaphragmatic or juxtadiaphragmatic schwannoma?]. / Schwannome diaphragmatique ou juxtadiaphragmatique?

The authors report one case of schwannoma of diaphragmatic topography. This lesion is exceptional and, to our knowledge, only seven cases have been published in the literature. The first diagnosis was a suprarenal tumour. Magnetic resonance imaging provided more detail about the juxta diaphragmatic topography of the tumour, which was subsequently confirmed by surgery. The diagnosis of benign schwannoma was made at the pathological and immuno-histochemical examination of the specimen. The schwannoma corresponded to the type B of the classification of Antoni. Pathogeny and origin of the tumour are discussed. The sympathetic nervous para-vertebral system, the phrenic or intercostal nerves could be the origin of the tumour. In the eight cases reported, the tumoural removal was performed through thoracotomy (n = 5) or laparotomy (n = 3). The preoperative exact location of the juxta diaphragmatic tumours remains difficult to specify.

[Pneumatosis cystoides of the colon. Apropos of 7 cases]. / Pneumatose kystique du côlon. A propos de 7 observations.

Seven cases of pneumatosis cystoides intestinalis affecting the colon (PKC) revealed 5 times by a nonspecific symtomatology and 2 times by an occlusion were reported. In three of the patients the disease was found as a primary idiopathic form free of clinical antecedents. In 4 of the patients the pneumatosis was found to be secondary to a pulmonary disease, a gastric ulcer, a connectivite of a corticotherapy. The PKC was generally diagnosed either by barium enema or by computed tomography and less frequently by colonoscopy with deep biopsy allowing differential diagnosis with colonic polyposis. The mechanism and etiology of the PKC were not fully understood. The illness is a benign condition that often responds to a conservative management--i.e. abstention, oxygenotherapy, diet or antibiotherapy- or exceptionally to surgical colonic resection in case of acute complication.