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Mastocytosis is a rare condition related to an abnormal proliferation of mast cells and their accumulation in tissues. Cutaneous mastocytosis is the most common form and mainly affects newborns and infants. The symptoms are caused by the release of mediators contained in mast cells, including histamine. Mastocytosis may be associated with a mutation in the gene encoding the c-kit receptor. Clinically, there are different dermatological findings, which combine acute cutaneous, digestive, or even hemodynamic manifestations in varying degrees. The diagnosis is confirmed by the histological study of a skin sample. We report here the case of a 4-month-old infant suffering from diffuse cutaneous bullous mastocytosis, a very rare variety of mastocytosis. This infant had an erosive and bullous manifestation of dermatosis, initially confused with impetigo. The proliferation of bullous lesions led to her hospitalization. Codeine intake for pain was responsible for a large and extensive bullous reaction associated with anaphylactic shock. This context of bullous spread occurring after taking codeine led to the suspicion of bullous diffuse cutaneous mastocytosis, a diagnosis that was confirmed histologically. This observation demonstrates the difficulty of mastocytosis diagnosis, mostly due to its rarity, especially in its diffuse bullous forms. The rapid deterioration of this patient, after the codeine prescription, emphasizes the importance of the eviction of histamine-releaser compounds in the management of this disease., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Unlabelled: Hepatic hemangioendothelioma can have a fatal outcome. After failure of classic therapies, and when surgery or arterial embolization are not possible, those benign tumours may benefit from interferon alpha treatment., Case Report: We report a case of a 14-month-old infant who presented with hepatic hemangiomatosis and cardiac failure. The disease can neither be controlled by steroids nor by radiotherapy associated with digitalo-diuretic treatment. Due to the important vascular volume of the tumour, neither surgical care nor arterial embolization were possible. Thus this infant was treated for ten months by interferon alpha. The evolution was favourable: cardiac failure, calcifications and volume of the angiomatosis were regressive., Conclusion: Interferon alpha treatment helps to control cardiac failure and the course of hepatic hemangioma in childhood.

This study presents the results of a hospital survey on Lyme disease in children living in upper Normandy, a region that is quite densely wooded (with 18% forest areas and woods). The aim of this survey was to assess the prevalence of this disease in children from the Seine-Maritime and L'Eure, hospitalized in pediatric wards in the Seine-Maritime department, which includes Rouen, Dieppe, Fécamp, Elbeuf, and Le Havre. Fifteen cases of Lyme disease were diagnosed between September 1988 and June 1997. The children (6 girls and 9 boys) were aged between 5 and 14 years old. Only 7 subjects showed primary symptoms, while secondary symptoms were observed in 12 children. In the study population, a high prevalence (11 out of the 15 children) of neurological disorders was found. The following secondary symptoms were noted: 5 cases of erythema migrans, 2 cases of non-malignant cutaneous lymphocytoma, and 4 cases which in fact had previously displayed primary clinical signs (3 subjects with erythema migrans and 1 subject with non-malignant cutaneous lymphocytoma); 7 cases of uni- or bilateral facial paralysis, the most frequent neurological manifestation with or without lymphocytic meningitis; 1 case of central vestibular syndrome with a hyperalgesic meningoradicular reaction in the vicinity of the tick bite; 1 case of peripheral radicular involvement and intense pain in the left lower limb; 4 cases of ocular disorders (3 diplopias, 1 bilateral conjunctivitis complicated by kerato-uveitis, 1 bilateral complete cecitis). Only 10 child had rheumatological symptoms, i.e., Lyme arthritis of the right knee. Treatment consisted of amoxicillin (10 children) administered at a dosage of 50 to 100 mg/kg/d over a period ranging from 10 days to 1 month, or ceftriaxone (7 children) at a dosage of 50 to 100 mg/kg/d administered intravenously over a period ranging from 8 days to 3 weeks. Two of the children received combined antibiotic therapy, and 5 subjects had adjunct corticotherapy.

Background: Benign infantile non febrile seizures are not well known, leading us to study their clinical and EEG characteristics., Methods: Between 1981 and 1994, we assembled 34 patients with the following inclusion criteria: non febrile seizures between 1 month and 2 years of age, normal personal history, no abnormality on clinical, biological and radiological investigations, normal developmental outcome with at least 1 year follow-up., Results: These 34 patients were recognized as 14 familial cases (identical seizures affecting parents) and 11 non familial cases. The other nine cases had different or undefined epilepsy in the family. The clinical and EEG characteristics were the same: at the mean age of 6 months, brief partial seizures (often secondarily or apparently generalized) occurring in a cluster of two to 12 episodes a day for a mean duration of 2.5 days, with ictal EEG showing focal discharge, often slow waves or focal spikes on post-ictal tracing and normal interictal EEG., Conclusion: The clinical and EEG characteristics are important in order to recognize this type of infantile convulsions (familial or not familial), which have a good prognosis and need no aggressive treatment.

Background: Neonatal myoclonic encephalopathy is of lesional or metabolic origin; non ketotic hyperglycinemia is one of its causes., Case Report: A girl, born from consanguineous parents, died from myoclonic epileptic encephalopathy at the age of 3 months. Screening for metabolic disease was negative, except for increased levels of urine serotonin and 5-hydroxyindol-acetic in cerebrospinal fluid, blood and urine. Two sisters died with non ketotic hyperglycinemia, corpus callosum agenesis and clubfoot., Conclusion: Familial occurrence of non ketotic hyperglycinemia and early myoclonic epileptic encephalopathy is uncommon.

Background: Prevalence of asthma is influenced by environmental factors which may be different from area to area., Population and Methods: A cross-sectional epidemiological survey was carried out among 1,395 children attending primary schools in the city of Le Havre and the canton of Fécamp (Haute-Normandie). Questionnaires including data about the disease, family history, environmental and socio-economic factors were completed by the school physician in presence of the parents., Results: Prevalence rate of asthma calculated from 1,193 questionnaires was 7.8%. The logistic regression curves pointed out four risk factors: family history of asthma (odds ratio: 2.52) or hay fever (OR: 1.98), atopic dermatitis (OR: 3.96), and parental smoking (OR: 1.79). Sex, socioeconomic status of parents, type of housing, presence of pets in the house were not related to prevalence of asthma. Frequency of cough during the day was only significantly different between both areas., Conclusions: The high level of school absence (21.1%) and frequency of hospitalizations for acute asthma (20.4%) should lead health professionals and educators to improve management of asthma in these areas.