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Introduction Les réactions d’hypersensibilité sont fréquentes lors de chimiothérapies par sels de platine ou taxanes, pouvant conduire à des arrêts prématurés de traitement. L’objectif était de montrer le caractère IgE médié ou non de ces réactions, et d’évaluer l’utilité et la tolérance des protocoles d’induction de tolérance (IDT) aux chimiothérapies. Méthodes Pour chaque patient adressé dans notre service suite à une réaction d’hypersensibilité survenue lors d’une chimiothérapie entre janvier 2007 et décembre 2014, le type et grade de réaction, les résultats des tests cutanés (prick-tests et intradermoréactions [IDR]), ainsi que le recours à une IDT et sa tolérance, étaient recueillis. Résultats Cinquante-six patients ayant présenté une hypersensibilité aux sels de platine ont été inclus : 51 avec une réaction immédiate (grade 1 : n = 24, grade 2 : n = 15, grade 3 : n = 11, grade 4 : n = 1) et 5 avec une réaction retardée. Six patients avaient des tests positifs pour l’oxaliplatine (prick-tests positifs : n = 3 ; IDR positives : n = 3). Six patients présentaient des tests positifs pour le carboplatine (prick-tests positifs : n = 1 ; IDR positives : n = 5). Aucun test n’était positif pour le cisplatine. Une IDT était proposée chez 19/56 patients, réalisée chez 10 d’entre eux dont 3 avaient des tests positifs. Outre les prémédications requises pour la chimiothérapie, un antihistaminique et une corticothérapie étaient systématiquement associés. L’IDT était bien tolérée chez 8/10 patients, dont 2 avec nécessité d’adaptation du protocole. Dix-huit patients ayant présenté une hypersensibilité aux taxanes ont été inclus : tous ont présenté une réaction immédiate (grade 1 : n = 4, grade 2 : n = 8, grade 3 : n = 6). Aucun test cutané n’était positif. Une IDT était proposée chez 4 patients, réalisée chez 2 d’entre eux avec une bonne tolérance. Une prémédication par antihistaminiques et corticothérapie était systématiquement associée. Conclusion Nous montrons que les protocoles d’IDT permettent de poursuivre la chimiothérapie avec succès pour la majorité des patients ayant présenté une hypersensibilité qu’elle soit IgE médiée ou non. Il s’agit d’une bonne alternative lorsque aucun autre traitement n’est possible. [ABSTRACT FROM AUTHOR]

Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an immunoglobulin G isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4 of 33 patients (12%) (smoldering myeloma, n = 2; chronic lymphoid leukemia, n = 1; and refractory cytopenia with multilineage dysplasia, n = 1). Carpal tunnel syndrome (33%), arthralgia (25%), and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. High-dose IV immunoglobulin (HDIVig), alone or in combination with steroids, appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4 of 5 patients. Quantitative reverse-transcriptase polymerase chain reaction analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor β and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management., (© 2020 by The American Society of Hematology.)

Background: Hair bleaching is increasingly being carried out in hairdressing salons. The products used are a mixture of hydrogen peroxide and persulfates, both active chemical agents. Scalp burns secondary to hair bleaching are a traumatic adverse effect rarely discussed in publications that continue to be little known among healthcare professionals., Patients and Methods: We report the case of a 15-year-old girl with a plaque of scarring alopecia on the vertex. This lesion resulted from a deep burn following a hair-bleaching procedure. Healing took around 4 months, resulting in discomfort for our patient., Discussion: This is a rare case of scarring alopecia following a basic chemical burn to the scalp. The oxidation reaction induced by the mixture of hydrogen peroxide and persulfates, prepared in a basic medium, causes bleaching of the melanin pigments in hair. The clinical presentation of a single, well limited, painful, oozing ulceration located at the vertex was similar to the other cases published in the literature. Although a chemical burning mechanism is most often incriminated, the procedure is always coupled with use of a heat source and associated thermal burn may occur. The delayed appearance of the lesion appears to be caused by the forming of surfactants by the hydrogen peroxide/persulfate mixture, resulting in slow dissolution of the oxidizing compounds within the stratum corneum., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Aim: The rate of hypersensitivity reactions to platinum salts (PS) and taxanes (TX) is on the increase. The aim of our study was to show the value of skin testing and efficacy of rapid drug desensitization., Patients and Methods: This was a retrospective study conducted between January 2007 and February 2016 in patients consulting for immediate or delayed hypersensitivity to PS and TX. Skin prick tests (pT) and intradermal reaction tests (IDR) were performed according to the ENDA/EAACI recommendations. We used a 12-step desensitization protocol for rapid drug desensitization., Results: Among the 99 patients included (30 men, 69 women, age 60.4) PS were suspected in 86 cases and taxanes in 13 cases. Skin tests were positive in 25 patients (7 pT, 18 IDR), 23 for platinum salts and 2 for taxanes. Rapid drug desensitization was proposed in 50 patients and performed in 33 (30 PS and 3 TX), proved effective in 29 patients, with protocol adaptation being necessary in 7 cases, and was ineffective in 4 patients. The skin tests for the latter 4 patients were positive. Seventy-five percent of patients with positive skin tests to oxaliplatin presented hypersensitivity reactions during desensitization, i.e. twice as many as patients having negative skin tests. Two percent of patient for PS and 7% for TX had cross reactivity., Conclusion: This French study confirms the efficacy of the 12-step protocol that allows patients to receive chemotherapy after hypersensitivity reaction. Skin test permits the detection of cross-reactions but their practice must be considered based on the patient's history., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: Livedoid vasculopathy (LV) is a painful ulcerative condition involving white atrophy and livedo; a histopathologic feature seen is occlusive dermal vasculopathy. This may be associated with coagulation disorders such as hyperhomocysteinaemia (HHC)., Patients and Methods: We report the case of a 52-year-old woman presenting LV in which an abnormal scan image led us to diagnose coeliac disease. This enteropathy had caused vitamin B12 and folic acid deficiency, as well as HHC. Vitamin supplementation and a gluten-free diet resulted in complete healing of the lesions., Discussion: This case underlines the importance of screening for and correction of coagulation disorders in patients with LV. It also suggests that in the event of HHC, coeliac disease should be sought, even in the absence of gastrointestinal symptoms., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background and Aims: Pictograms, designed to be a universal communication system, are often created from several concrete and easily recognizable drawings. Does understanding depend on a logical approach? Or is it the ability to inhibit the concrete sense of each picture that allows access to a higher level of comprehension? (ability to abstract). These executive functions are sensitive to the effects of aging and educational level. The aim of our study was to evaluate the nature of the cognitive processes underlying the meaning of pictograms and to test the effect of aging and educational level., Methods: We enrolled 19 older adults (60-69 years old) and 63 young adults (20-29 years old). Of these 63 young adults, 43 had a high educational level (Young-High participants), and 20 had a lower educational level (Young-Low participants). Each participant was asked the meaning of 20 pictograms and underwent an assessment of abstraction and logical abilities with WAIS-III test., Results: Older adults had lower pictogram assessment scores and abstraction and logical abilities when compared with young adults. In both groups, abstraction and logical abilities were correlated with the interpretation of pictograms but only abstraction ability remains strongly correlated with pictogram comprehension in the older group after adjustment of sex, age and educational level. Consequently, the poorer performances of older adults to determine the meaning of pictograms could be explained by the decline of abstraction ability in elderly., Conclusions: Pictograms are not the universal communication system as we formerly thought. Age and educational level may influence the performance in determining the meaning of pictograms.

Unlabelled: Methylphenidate (MPH) is a potential therapeutic tool for Attention Deficit with Hyperactivity Disorders (ADHD). In addition to the immediate-release formulation, Ritalin, two extended-release formulations, Ritalin LA and Concerta are available and allow a once-daily administration. We compared the respective benefits of both formulations for the patients and their family in terms of efficacy, handling and tolerance., Patients and Methods: This prospective study was based on 30 children aged 6 to 15 years. All patients had a confirmed ADHD and were efficiently treated with Ritalin. The children were consecutively treated with Ritalin LA and Concerta, with a comparable MPH daily dosage, during 2 months for each molecule. The 3 drugs were evaluated individually and comparatively through a battery of questionnaires submitted to the parents and the teachers of each child., Results: Extended-release MPH efficacy was comparable to the immediate-release formulation, Ritalin. For both of them, the once-daily administration appeared beneficial. Concerta was finally prescribed in 18 children, Ritalin LA in 8 cases and Ritalin in 4 cases. In each case the medical choice was consistent with the parents preference. Concerta was appreciated for its persisting efficacy in late afternoon during homework. Concerta and Ritalin LA did not induce significant adverse effects, especially regarding alimentation and sleep., Conclusions: MPH therapy in ADHD carries an excellent risk/benefit ratio without addictive induced behaviours. The extended-release MPH formulations provide an improvement for the patients in keeping with Ritalin efficacy through a once-daily administration. Regardless of its formulation, MPH indications and guidelines must be respected.

Purpose: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike-waves during slow sleep (CSWS) and Landau-Kleffner syndromes, which are two rare epileptic syndromes occurring in children., Methods: We enrolled seven young adults, five who had a CSWS syndrome, and two, a Landau-Kleffner syndrome in childhood. We evaluated their intellectual level as well as their oral and written language and executive functions., Results: This study confirmed that the epilepsy associated with these syndromes has a good prognosis. Only one patient still had active epilepsy. However, the neuropsychological disorders particular to each syndrome persisted. Only two patients had followed a normal pathway in school. Three of the five patients with a CSWS syndrome during childhood remained globally and nonselectively mentally deficient. We found no evidence of the persistence of a dysexecutive syndrome in this study group. The intellectual functions of the two patients with Landau-Kleffner syndrome were normal; however, their everyday lives were disrupted by severe, disabling language disturbances. We discuss the role of some prognostic factors such as the location of the interictal electric focus and the age at onset of CSWS., Conclusions: These two epileptic syndromes of childhood are very similar in many respects, but their clinical outcomes in adulthood are different.