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Introduction: Poems syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin lesions., Case: We describe here a case in which the eruption of diffuse cutaneous angiomas in a woman with a history of bone plasmocytoma and progressive polyneuropathy helped physicians to diagnose Poems syndrome., Discussion: Other manifestations of Poems syndrome in this patient included endocrine dysfunction (hypothyroidism, adrenal insufficiency, and hypogonadism), sclerotic bone lesions of the femoral shaft, ribs, and vertebral body, monoclonal gammopathy, and anasarca. Steroid treatment led to dramatic improvement of polyserositis and a generally good outcome, despite persistence of the cutaneous lesions. This case points out this unusual cutaneous manifestation associated with Poems syndrome.

Unlabelled: This observation recalls that gastric phytobezoar should lead to a search for an underlying disease and that a iron deficiency can be associated and hide macrocytosis related to a vitamin B12 deficiency., Case Report: A 19 year-old woman consulted for asthenia. Microcyte anaemia associated with iron deficiency was diagnosed. Upper digestive endoscopy revealed severe, totally asymptomatic phytobezoar. Biological investigations revealed a vitamin B12 deficiency, high serum gastrin level and strong positivity for gastric antiparietal anti-cell antibodies, suggestive of an autoimmune gastritis. Total immunoglobulin A deficiency was also noted., Discussion: Autoimmune gastritis is responsible for megaloblastic anaemia (vitamin B12 deficiency) but can also provoke microcytic (iron-deficiency) anaemia due to insufficient absorption of the latter and related to gastric achlorhydria. Phytobezoar might also be related to achlorhydria and/or gastroparesia associated with autoimmune gastritis. Hence, autoimmune gastritis should be searched for when confronted with unexplained gastric bezoar or iron-deficiency anaemia.

Introduction: Down's syndrome is characterized by an abnormal frequency of coeliac disease and by the frequent occurrence of neurological disorders such as Alzheimer-type dementia of early onset. However, psychosis is rare in Down's syndrome., Observation: We report the case of a 41-year-old woman, who presented with Down's syndrome. She was living with her parents and had a normal social life. She suddenly experienced some esthesic hallucinations, depression, anorexia, affective flattening and autistic behavior. Biological evaluation revealed macrocytosis, polyclonal IgA and IgG hypergammaglobulinemia and strong positivity for anti-gliadin antibodies of IgG and IgA isotypes. Brain CTscan was normal. Since digestive specimen biopsies did not evidence villous atrophy, we concluded in a silent coeliac disease. After 12 months of gluten-free diet a spectacular and lasting improvement of both psychotic and depressive symptoms was obtained., Discussion: The effects of abnormal interaction between the immune system and gluten can be expressed not only in the gut (coeliac disease) but also in the brain (psychosis) in genetically predisposed patients, such as those suffering from Down's syndrome. There was evidence for brain disorder related to coeliac disease in our patient., Conclusion: Our case report shows that, before concluding in Alzheimer-type dementia in Down's syndrome, a biological search for coeliac disease is useful since a gluten-free diet may improve the psychiatric symptoms.

Objective: Heyde's syndrome associates aortic stenosis and digestive hemorrhage secondary to intestinal angiodysplasia., Observation: A 61 year-old man presented with anemia due to martial deficiency and melena, the endoscopic examination of which was negative. The existence of a tight aortic stenosis, suspected on auscultation, suggested the diagnosis of Heyde's syndrome. Angiodysplasia is revealed during oriented enteroscopy. Following valve replacement, the need for transfusion disappeared, together with the anemia., Discussion: Although some epidemiological studies deny the existence of this syndrome, the observation of authentic cases of resolution of digestive bleeding following surgical replacement of an aortic valve, such as ours, is an argument in favor of the reality of Heyde's syndrome.

Background: Sicca syndrome after Stevens-Johnson syndrome is classically described. However, to our knowledge, authentic Sjögren's syndrome following epidermal necrolysis has not been described previously., Case Report: A 36-year-old woman with Steven-Johnson syndrome developed transient hepatitis and a persistent sicca syndrome. Fourteen years later an authentic Sjögren's syndrome was identified with presence of antinuclear and anti-SSA antibodies and lymphocytes infiltration of the accessory salivary glands, i.e. grade IV disease in the Chisholm classification., Discussion: The initial mucosal destruction observed in our patients may have laid the ground for Sjögren's syndrome via abnormal antigen presentation in a predisposed dysimmune context.

Background: Primary periodic palsy is a group of muscular diseases transmitted by autosomal dominant inheritance. The characteristic features are flaccid muscular deficiency, abolition of reflexes, and dyskalemia., Case Report: A 36-year-old woman presented a 5-year history of acute episodes of myalgia, muscle contracture, and muscle paralysis involving first the hand, then all four limbs and the face, sparing the respiratory muscles. The symptoms totally regressed spontaneously within one hour or in a shorter time if the patient ingested sugar. Demonstration of hyperkalemia during an acute episode led to the diagnosis of transfer hyperkalemia typical of Gamstorp's disease. Several members of the family presented identical symptoms., Discussion: Despite progress in our understanding of this disease, diagnosis is often made late. The diagnostic strategy is however quite simple: serum potassium during an acute episode and provocation test with fasting and rest.

Background: The underlying cause of camptocormia, a common condition in adults consisting of forward flexion of the trunk both in stance and gait, is often unrecognized, attributed by some to primary late-onset myopathy., Case Reports: Two patients progressively developed camptocormia. The cause was identified as a relatively new disease, proximal myotonic myopathy., Discussion: The diagnosis of proximal myotonic myopathy is based on clinical findings: myalgia and diffuse pain, gait disorders and progressive weakness of proximal muscles, electrical myotonia, cataracte in some cases, elevated gamma glutamyltranspeptidases. The disease is transmitted by autosomal dominant inheritance. This condition should be entertained as a possible cause in patients presenting an apparently primary camptocormic syndrome.

Background: Behçet's disease is an uncommon cause of prolonged fever. This misleading presentation is illustrated by a new case where fever occurred after polyvalent vaccination., Case Report: A 39-year-old female patient was seen for prolonged fever after returning from a trip to Africa. She had received polyvalent vaccinations prior to leaving France. At admission fever had persisted for 2 months. The diagnosis of Behçet's disease was finally established on the basis of clinical signs, buccogenital aphtosis and pathergy. Colchicine was partially effective against although low-grade persisted in the evening., Discussion: Clinicians should be aware that Behçet's disease can provoke long-term fever which can sometimes be an inaugural sign. Sensitivity tests may be useful to identify the trigger, for example polyvalent vaccination.