[Eosinophilia heralding the diagnosis of eosinophilic fasciitis (Shulman's disease)]. / Hyperéosinophilie isolée précédant le diagnostic d'une fasciite à éosinophiles (maladie de Shulman).

INTRODUCTION: Eosinophilic fasciitis or Shulman's disease is characterized, in its typical form, by palpable thickening of the skin and soft tissues, blood hypereosinophilia and fascia lesions. We hereby report a case of eosinophilic fasciitis in which hypereosinophilia preceded for several months the clinical signs of fasciitis. CASE REPORT: A 64-year-old woman, with a history of Little's syndrome with motor disability, was admitted in internal medicine for eosinophilia. For almost three months, no origin to the eosinophilia was found. The secondary onset of an edema and pain located on four limbs led to the diagnosis of eosinophilic fasciitis. Muscle magnetic resonance imaging was supportive and the muscle histological analysis confirmed the diagnosis of eosinophilic fasciitis. Treatment with steroids induced a rapid normalization of the eosinophilia and edema. CONCLUSION: In this case report, eosinophilia was preceding the clinical cutaneous signs that led to the diagnosis of eosinophilic fasciitis. It is likely to believe that myalgias, frequently found in the onset of eosinophilic fasciitis, may have been hidden by the history of infantile encephalopathy. The diagnosis of eosinophilic fasciitis must be kept in mind of physicians in the investigation of an eosinophilia, even though cutaneous signs are lacking.

State of the art. Autologous fat graft and adipose tissue-derived stromal vascular fraction injection for hand therapy in systemic sclerosis patients.

Systemic sclerosis is an autoimmune disease characterized by sclerosis (hardening) of the skin and deep viscera associated with microvascular functional and structural alteration, which leads to chronic ischemia. In the hands of patients, ischemic and fibrotic damages lead to both pain and functional impairment. Hand disability creates a large burden in professional and daily activities, with social and psychological consequences. Currently, the proposed therapeutic options for hands rely mainly on hygienic measures, vasodilatator drugs and physiotherapy, but have many constraints and limited effects. Developing an innovative therapeutic approach is crucial to reduce symptoms and improve the quality of life. The discovery of adult stem cells from adipose tissue has increased the interest to use adipose tissue in plastic and regenerative surgery. Prepared as freshly isolated cells for immediate autologous transplantation, adipose tissue-derived stem cell therapy has emerged as a therapeutic alternative for the regeneration and repair of damaged tissues. We aim to update literature in the interest of autologous fat graft or adipose derived from stromal vascular fraction cell-based therapy for the hands of patients who suffer from systemic sclerosis.

[Cannabinoid hyperemesis syndrome: about 6 cases]. / Syndrome d'hyperémèse cannabique : à propos de 6 cas.

INTRODUCTION: The cannabinoid hyperemesis syndrome (CHS) is characterized by cyclic episodes of nausea, vomiting, and abdominal pain, and occurs in young adults with long-term cannabis use. The feature of this syndrome is the relief of symptoms with hot showers. We report here six cases report. CASE REPORTS: Three women and 3 men, chronic consumers of cannabis, presented with typical features of the CHS, but the syndrome remained undiagnosed until then: nausea, vomiting, abdominal pain with morning ascendancy and loss of weight occurring in adult's of less than 50 years. The symptoms were improved by taking repeated hot showers. The medical investigations were negative. After addictologist care and cannabis weaning, digestive symptoms disappeared. CONCLUSION: The CHS is based on a clinical diagnosis. When undiagnosed, clinical presentation leads to a medical wandering and to the realization of repeated, expensive, and sometimes invasive exams. The physiopathology is not clear. The treatment relies on the definitive cannabis weaning.

[Valproate-induced hyperammonemic encephalopathy]. / Encéphalopathie hyperammoniémique au valproate de sodium.

Valproate is a drug commonly prescribed in neurology and psychiatry. Hyperammonemic encephalopathy due to valproate is a severe complication. A 44-year-old man with a past medical history of partial symptomatic epilepsy and alcoholic cirrhosis was admitted for drowsiness. He was receiving valproate and gabapentin. A valproate-induced hyperammonemic encephalopathy was diagnosed on physical examination, generalized slow waves on electroencephalogram and hyperammonemia. Valproate withdrawal led to a progressive recovery of the consciousness, with a rapid normalisation of electroencephalogram and ammonium level. The pathogenesis of this encephalopathy is not clearly established. No correlation has been shown between the severity of encephalopathy, the plasma ammonium level, the valproate dose and its plasma concentration. Additional factors have been pointed out, such as carnitine deficiency or urea cycle enzyme defects. Furthermore, our case suggests an enhancing role of the liver disease in this encephalopathy.