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1.
J Eur Acad Dermatol Venereol ; 36(12): 2423-2429, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35854650

RESUMO

BACKGROUND: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD). OBJECTIVE: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice. METHODS: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial. RESULTS: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial. LIMITATIONS: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information. CONCLUSION: These real-life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group.


Assuntos
Conjuntivite , Dermatite Atópica , Criança , Humanos , Dermatite Atópica/tratamento farmacológico , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Conjuntivite/induzido quimicamente , Estudos de Coortes , Imunoglobulina A
2.
J Eur Acad Dermatol Venereol ; 36(11): 2076-2086, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35748102

RESUMO

BACKGROUND: The COVID-19 pandemic has raised questions regarding the management of chronic skin diseases, especially in patients on systemic treatments. Data concerning the use of biologics in adults with psoriasis are reassuring, but data specific to children are missing. Moreover, COVID-19 could impact the course of psoriasis in children. OBJECTIVES: The aim of this study was therefore to assess the impact of COVID-19 on the psoriasis of children, and the severity of the infection in relation to systemic treatments. METHODS: We set up an international registry of paediatric psoriasis patients. Children were included if they were under 18 years of age, had a history of psoriasis, or developed it within 1 month of COVID-19 and had COVID-19 with or without symptoms. RESULTS: One hundred and twenty episodes of COVID-19 in 117 children (mean age: 12.4 years) were reported. The main clinical form of psoriasis was plaque type (69.4%). Most children were without systemic treatment (54.2%); 33 (28.3%) were on biologic therapies, and 24 (20%) on non-biologic systemic drugs. COVID-19 was confirmed in 106 children (88.3%) and 3 children had two COVID-19 infections each. COVID-19 was symptomatic for 75 children (62.5%) with a mean duration of 6.5 days, significantly longer for children on non-biologic systemic treatments (P = 0.02) and without systemic treatment (P = 0.006) when compared with children on biologics. The six children who required hospitalization were more frequently under non-biologic systemic treatment when compared with the other children (P = 0.01), and particularly under methotrexate (P = 0.03). After COVID-19, the psoriasis worsened in 17 cases (15.2%). Nine children (8%) developed a psoriasis in the month following COVID-19, mainly a guttate form (P = 0.01). DISCUSSION: Biologics appear to be safe with no increased risk of severe form of COVID-19 in children with psoriasis. COVID-19 was responsible for the development of psoriasis or the worsening of a known psoriasis for some children.


Assuntos
Produtos Biológicos , COVID-19 , Psoríase , Adolescente , Adulto , Fatores Biológicos/uso terapêutico , Produtos Biológicos/uso terapêutico , COVID-19/complicações , Criança , Progressão da Doença , Humanos , Metotrexato/uso terapêutico , Pandemias , Psoríase/complicações , Psoríase/tratamento farmacológico , Psoríase/epidemiologia , Sistema de Registros
5.
J Eur Acad Dermatol Venereol ; 35(11): 2263-2269, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34273203

RESUMO

BACKGROUND: Atopic dermatitis (AD) is a chronic, relapsing, inflammatory skin disease. Therapeutic patient education (TPE) has been demonstrated to be effective in AD in reducing disease severity and improving coping and quality of life. OBJECTIVES: To describe the sociodemographic and clinical characteristics of children and adolescents with AD who had attended TPE sessions, as well as the characteristics of their parents, and compare them with those who did not attend TPE. METHODS: Parents of children with AD aged 6-17 years old were recruited from a representative sample of the French population contacted by e-mail. Sociodemographic data and clinical information were collected in patients and parents. Clinical severity was assessed by parents using a proxy version of the Patient-Oriented Eczema Measure (POEM). Attendance to TPE sessions was assessed by the following question 'did your child or one or both parents attended TPE for AD?'. Also, the number of sessions was recorded. Determinants of TPE attendance were evaluated by univariable and multivariable analyses. RESULTS: Data were collected on 1063 parents and children with AD. A total of 131 (12.3%) children and/or parents attended TPE sessions. Most of them attended 2-5 TPE sessions. In that group, there were 85 boys (64.9%), and severity evaluated by POEM was mild in 29.8%, moderate in 52.7% and severe in 17.6% of patients. In the multivariable model, attending TPE sessions was significantly associated with sex of the child (boy vs. girl), consultation with a dermatologist or a paediatrician, high clinical severity and presence of AD in parents. CONCLUSIONS: Despite recommendations, the use of TPE in children with AD is still low in France. There is a need for implementing such programmes in the management of the disease, in particular when the disease is severe.


Assuntos
Dermatite Atópica , Eczema , Adolescente , Criança , Dermatite Atópica/terapia , Feminino , Humanos , Masculino , Pais , Educação de Pacientes como Assunto , Qualidade de Vida , Índice de Gravidade de Doença
6.
Ann Dermatol Venereol ; 148(2): 94-100, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33551211

RESUMO

BACKGROUND: A marked increase in frequency of acute acral eruptions (AAE) was observed in children during the COVID-19 pandemic in the spring period. OBJECTIVES: In this observational multicenter study, based on children with AAE, we aimed to assess the proportion of household members possibly infected by SARS-CoV-2. METHODS: We collected data from all children observed with AAE, prospectively from April 7, 2020 to June 22, 2020, and retrospectively since February 28, 2020. The primary outcome was the household infection rate, defined as the proportion of family clusters having at least one member with COVID-19 infection other than the child with AAE ("index child"). The definition of a case was based on characteristic clinical signs and a positive PCR or serology. RESULTS: The study included 103 children in 10 French departments and in Quebec. The median age was 13 years and the interquartile range [8-15], with a female-to-male ratio of 1/1.15. In children with AAE, all PCR tests were negative (n=18), and serology was positive in 2/14 (14.3%) cases. We found no significant anomalies in the lab results. A total of 66 of the 103 families (64.1%) of included children had at least one other infected member apart from the index child. The total number of household members was 292, of whom 119 (40.8%) were considered possibly infected by SARS-CoV-2. No index children or households exhibited severe COVID-19. DISCUSSION: Among the 103 households included, 64.1% had at least one infected member. Neither children with AAE nor their households showed severe COVID-19.


Assuntos
COVID-19/complicações , Família , Adolescente , Anticorpos Antinucleares/sangue , COVID-19/transmissão , Pérnio/patologia , Criança , Eritema/patologia , Feminino , Hidradenite/patologia , Humanos , Imunoglobulina G/sangue , Linfócitos/patologia , Masculino , Mucinoses/patologia , Pandemias , Estudos Retrospectivos , Pele/patologia , Vasculite/patologia
8.
Ann Dermatol Venereol ; 147(5): 361-365, 2020 May.
Artigo em Francês | MEDLINE | ID: mdl-32164923

RESUMO

BACKGROUND: Apocrine hidrocystomas are benign cystic tumors that develop from apocrine gland proliferation. In most cases, they are translucent solitary lesions of the face, generally found in the periorbital region, on the scalp or on the neck. More rarely, apocrine hidrocystomas may be multiple and appear on the ears, trunk, shoulders and genital area. They generally appear in adulthood, with only a few pediatric cases being reported, of which three in the genital area, with a solitary case of multiple hidrocystomas of the scrotum, although no cases of spontaneous involution of hidrocystomas have previously been reported. PATIENTS AND METHODS: Two boys aged 4 and 6 months were seen in consultation for small sub-millimeter size, subcutaneous, black lesions on the scrotum that appeared in the weeks following birth. Histological examination of these lesions resulted in a diagnosis of apocrine hidrocystoma. The children were seen again a few weeks later and the skin lesions had totally disappeared. We report two cases of multiple apocrine hidrocystomas on the scrotum with spontaneous involution diagnosed in a 4- and a 6-month-old boy. DISCUSSION: Apocrine hidrocystomas are rare benign adnexal tumors that develop from apocrine sweat glands. They are considered as cystic proliferations of the apocrine glands rather than simple retention cysts. The main differential diagnosis of the rare cases of multiple apocrine hidrocystomas are eccrine hidrocystomas. The treatment of such lesions is based on surgical excision if they are isolated, daily application of topical atropine 1%, or CO2 laser for multiple apocrine hidrocystomas.


Assuntos
Glândulas Apócrinas , Neoplasias dos Genitais Masculinos/patologia , Hidrocistoma/patologia , Escroto , Neoplasias das Glândulas Sudoríparas/patologia , Humanos , Lactente , Masculino
9.
J Eur Acad Dermatol Venereol ; 33(10): 1984-1992, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30883928

RESUMO

BACKGROUND: Three biotherapies - etanercept, adalimumab and ustekinumab - are licensed in childhood psoriasis. The few data available on their efficacy and tolerance are mainly derived from industry trials. However, biological drug survival impacts long-term performance in real-life settings. OBJECTIVE: The objective of this study was to evaluate the survival rates of biological therapies in children with psoriasis in real-life conditions. Secondary objectives were to evaluate the factors associated with the choice of the biological therapy and to report severe adverse events. MATERIALS AND METHODS: This study was an observational retrospective study. Data were extracted from the clinical records of 134 children. Kaplan-Meier estimates were used to analyse drug survival overall and in subgroups of plaque psoriasis, bio-naïve and non-naïve patients. RESULTS: We analysed 184 treatment courses: 70 with etanercept, 68 with adalimumab and 46 with ustekinumab. Factors associated with the choice of first-line biological agent were age at initiation (younger for adalimumab, P < 0.0001), age at onset of psoriasis (younger for adalimumab and etanercept, P = 0.03) and baseline Psoriasis Assessment Severity Index and Physician global assessment (both higher for adalimumab, P < 0.001). Drug survival rates were higher for ustekinumab than for adalimumab and etanercept (P < 0.0001) for all treatment and all psoriasis types, plaque-type psoriasis (P = 0.0003), patients naïve for biological agents (P = 0.0007) and non-naïve patients (P = 0.007). We reported eight serious adverse events (SAEs): severe infections (n = 3), significant weight gain (n = 2), psoriasis flare (n = 1) and malaise (n = 1). Biological therapy was discontinued in three children (one with psoriasis flare and two with weight gain). Only the two cases of weight gain resulted in an unfavourable outcome. CONCLUSIONS: Our real-life comparative study found that ustekinumab had the best drug survival outcome. The profile of SAEs in children was comparable to that in adults. These results will assist dermatologists in the decision-making process when choosing treatment options for children with psoriasis in daily practice.


Assuntos
Adalimumab/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Etanercepte/uso terapêutico , Psoríase/tratamento farmacológico , Ustekinumab/uso terapêutico , Adalimumab/efeitos adversos , Adolescente , Fatores Etários , Produtos Biológicos/uso terapêutico , Criança , Tomada de Decisão Clínica , Fármacos Dermatológicos/efeitos adversos , Etanercepte/efeitos adversos , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Adesão à Medicação , Estudos Retrospectivos , Índice de Gravidade de Doença , Ustekinumab/efeitos adversos
11.
Genes Brain Behav ; 16(7): 699-708, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28398003

RESUMO

Midkine (MDK) is a cytokine and neurotrophic factor that is more highly expressed in the brains of alcoholics and in mice predisposed to drink large amounts of ethanol, suggesting that MDK may regulate ethanol consumption. Here we measured ethanol consumption in male and female Mdk knockout (-/-) mice using the two-bottle choice and the drinking in the dark (DID) tests. We found that Mdk -/- mice consumed significantly more ethanol than wild-type controls in both tests. To determine if MDK acts in the ventral tegmental area (VTA) to regulate ethanol consumption, we delivered lentivirus expressing a Mdk shRNA into the VTA of male C57BL/6J mice to locally knockdown Mdk and performed the DID test. Mice expressing a Mdk shRNA in the VTA consumed more ethanol than mice expressing a control non-targeting shRNA, demonstrating that the VTA is one site in the brain through which MDK acts to regulate ethanol consumption. Since MDK also controls the expression of inflammatory cytokines in other organs, we examined gene expression of interleukin-1 beta (Il1b), tumor necrosis factor alpha (Tnfα) and the chemokine (C-C motif) ligand 2 (Ccl2) in the VTA of Mdk -/- mice and in mice expressing Mdk shRNA in the VTA. Expression of Ccl2 was elevated in the VTA of Mdk -/- mice and in mice expressing Mdk shRNA in the VTA. These results demonstrate that MDK functions in the VTA to limit ethanol consumption and levels of CCL2, a chemokine known to increase ethanol consumption.


Assuntos
Consumo de Bebidas Alcoólicas/genética , Quimiocina CCL2/metabolismo , Peptídeos e Proteínas de Sinalização Intercelular/metabolismo , Área Tegmentar Ventral/metabolismo , Consumo de Bebidas Alcoólicas/metabolismo , Animais , Quimiocina CCL2/genética , Peptídeos e Proteínas de Sinalização Intercelular/genética , Interleucina-1beta/genética , Interleucina-1beta/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Midkina , Fator de Necrose Tumoral alfa/genética , Fator de Necrose Tumoral alfa/metabolismo , Área Tegmentar Ventral/fisiologia
13.
Transl Psychiatry ; 6(11): e953, 2016 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-27845775

RESUMO

The corticotropin releasing factor (CRF) exerts its effects by acting on its receptors and on the binding protein (CRFBP), and has been implicated in alcohol use disorder (AUD). Therefore, identification of the exact contribution of each protein that mediates CRF effects is necessary to design effective therapeutic strategies for AUD. A series of in vitro/in vivo experiments across different species were performed to define the biological discrete role of CRFBP in AUD. First, to establish the CRFBP role in receptor signaling, we developed a novel chimeric cell-based assay and showed that CFRBP full length can stably be expressed on the plasma membrane. We discovered that only CRFBP(10 kD) fragment is able to potentiate CRF-intracellular Ca2+ release. We provide evidence that CRHBP gene loss increased ethanol consumption in mice. Then, we demonstrate that selective reduction of CRHBP expression in the center nucleus of the amygdala (CeA) decreases ethanol consumption in ethanol-dependent rats. CRFBP amygdalar downregulation, however, does not attenuate yohimbine-induced ethanol self-administration. This effect was associated with decreased hemodynamic brain activity in the CRFBP-downregulated CeA and increased hemodynamic activity in the caudate putamen during yohimbine administration. Finally, in alcohol-dependent patients, genetic variants related to the CRFBP(10 kD) fragment were associated with greater risk for alcoholism and anxiety, while other genetic variants were associated with reduced risk for anxiety. Taken together, our data provide evidence that CRFBP may possess both inhibitory and excitatory roles and may represent a novel pharmacological target for the treatment of AUD.


Assuntos
Consumo de Bebidas Alcoólicas/genética , Alcoolismo/genética , Proteínas de Transporte/genética , Consumo de Bebidas Alcoólicas/fisiopatologia , Alcoolismo/fisiopatologia , Tonsila do Cerebelo/fisiopatologia , Animais , Cálcio/metabolismo , Membrana Celular/metabolismo , Regulação para Baixo/genética , Expressão Gênica/genética , Humanos , Sistema Hipotálamo-Hipofisário/fisiopatologia , Imageamento por Ressonância Magnética , Masculino , Camundongos , Camundongos Endogâmicos , Camundongos Knockout , Sistema Hipófise-Suprarrenal/fisiopatologia , Fluxo Sanguíneo Regional/genética , Especificidade da Espécie , Adulto Jovem
14.
Br J Dermatol ; 175(4): 776-81, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27037896

RESUMO

BACKGROUND: Anti-p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin γ1. The clinical course of anti-p200 pemphigoid in patients remains poorly investigated. OBJECTIVES: We aimed to describe the clinical and immunological features and the course of a series of patients with anti-p200 pemphigoid. METHODS: We conducted a retrospective study by immunoblotting detection of sera on 200-kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients. RESULTS: A total of 14 patients with a mean age 81·6 ± 6·5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including eczematous, urticarial, prurigo-like, dyshydrosis-like and rosette-like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200-kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme-linked immunosorbent assay detected recombinant human laminin γ1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow-up time of 12·6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy. CONCLUSIONS: Many patients with anti-p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected.


Assuntos
Laminina/imunologia , Penfigoide Bolhoso/patologia , Idoso , Idoso de 80 Anos ou mais , Fármacos Dermatológicos/uso terapêutico , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/imunologia , Prognóstico , Recidiva , Estudos Retrospectivos
16.
Genes Brain Behav ; 13(8): 777-83, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25176312

RESUMO

LIM-domain-only 3 (LMO3) is a transcriptional regulator involved in central nervous system development and neuroblastoma. Our previous studies implicated a potential role for LMO3 in regulating ethanol sensitivity and consumption. Here, we examined behavioral responses to ethanol in a line of Lmo3 null (Lmo3(Z) ) mice, utilizing the ethanol-induced loss-of-righting-reflex (LORR) test, two-bottle choice ethanol consumption and the drinking in the dark (DID) test, which models binge-like ethanol consumption. We found that Lmo3(Z) mice exhibited increased sedation time in response to ethanol in the LORR test and drank significantly more ethanol in the DID test compared with their wild-type counterparts, but showed no differences in two-bottle choice ethanol consumption. To explore where LMO3 may be acting in the brain to produce an ethanol phenotype, we also examined reporter gene (ß-galactosidase) expression in heterozygous Lmo3(Z) mice and found strong expression in subcortical areas, particularly in those areas implicated in drug abuse, including the nucleus accumbens (Acb), cortex, hippocampus and amygdala. We also examined Lmo3 expression in the brains of wild-type mice who had undergone the DID test and found a negative correlation between Lmo3 expression in the Acb and the amount of ethanol consumed, consistent with the increased binge-like drinking observed in Lmo3(Z) mice. These results support a role for LMO3 in regulating behavioral responses to ethanol, potentially through its actions in the Acb.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/genética , Consumo de Bebidas Alcoólicas/genética , Comportamento Animal/efeitos dos fármacos , Encéfalo/metabolismo , Etanol/farmacologia , Proteínas com Domínio LIM/genética , Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Consumo de Bebidas Alcoólicas/metabolismo , Animais , Comportamento Animal/fisiologia , Encéfalo/efeitos dos fármacos , Comportamento de Escolha/efeitos dos fármacos , Comportamento de Escolha/fisiologia , Proteínas com Domínio LIM/metabolismo , Camundongos , Camundongos Knockout
17.
Ann Dermatol Venereol ; 141(3): 215-8, 2014 Mar.
Artigo em Francês | MEDLINE | ID: mdl-24635957

RESUMO

BACKGROUND: Herein we report the first case of toxic epidermal necrolysis (TEN) occurring with use of vemurafenib. PATIENTS AND METHODS: A 75-year-old female patient was being treated with vemurafenib for stage IV melanoma with BRAF V600E mutation. She suddenly presented fever, diffuse pruriginous maculopapular erythema, palpebral edema, palmar bulla, conjunctivitis, cheilitis and mucosal ulceration. The condition progressed towards detachment affecting 50% of the skin area. Cutaneous biopsy revealed lichenoid dermatosis, chiefly vesicular with numerous eosinophils. Direct immunofluorescence (IFD) was negative. Vemurafenib was the only drug to which the reaction was ascribable and we concluded on vemurafenib-induced TEN. DISCUSSION: To our knowledge, this is the first reported case of vemurafenib-induced TEN, but this adverse effect, although already described in the BRIM-3 study, appears rare in clinical practice. Other severe skin reactions have been described in the literature. These include a case of Stevens-Johnson syndrome in a female patient treated with vemurafenib and previously receiving ipilimumab. A more common occurrence is cutaneous reactions involving efflorescence of benign hyperkeratotic lesions, occasionally accompanied by authentic epidermal carcinoma or keratoacanthoma, and requiring regular dermatological monitoring of patients treated with vemurafenib. CONCLUSION: If maculopapular exanthema occurs under vemurafenib, continuation of this treatment should be reassessed since the risk of progression to a more serious condition such as TEN, as seen in the present case, cannot be ruled out.


Assuntos
Indóis/efeitos adversos , Melanoma/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Síndrome de Stevens-Johnson/etiologia , Sulfonamidas/efeitos adversos , Idoso , Biópsia , Feminino , Humanos , Indóis/uso terapêutico , Melanoma/genética , Melanoma/patologia , Mutação , Estadiamento de Neoplasias , Proteínas Proto-Oncogênicas B-raf/genética , Pele/patologia , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/patologia , Sulfonamidas/uso terapêutico , Vemurafenib
18.
Arch Pediatr ; 21(3): 299-301, 2014 Mar.
Artigo em Francês | MEDLINE | ID: mdl-24468061

RESUMO

Erythermalgia is a peripheral vascular disease triggered by exposure to heat. The primary infantile form is rare. No cases have been described in infants. We report a case in a 6-month-old child revealed by crying bouts associated with erythema of the lower limbs. A 6-month-old child was brought in for consultation for daily crying bouts, occurring six times a day, associated with erythema of the lower limbs. Blood count, abdominal ultrasound and endoscopy were normal, excluding gastroesophageal reflux and intussusception. Attacks disappeared during winter but recurred at high temperatures. The diagnosis was primary infant erythemalgia. Treatment with analgesics and ice packs was established. Erythermalgia is a rare peripheral vascular disease characterized by paroxysmal pain triggered by heat and relieved by cold. The primary form occurs in childhood but has never been reported in infants. The pathophysiology is based on an alteration of sodium channels inducing neuropathy in small-caliber fibers. Genetic mutations have been found in the SNC9 gene on chromosome 2q, with autosomal dominant transmission. Support of this condition is difficult due to resistance to conventional analgesics. The prognosis is sometimes poor with a significant death rate in the pediatric population.


Assuntos
Choro , Eritromelalgia/diagnóstico , Humanos , Lactente , Masculino
19.
Ann Dermatol Venereol ; 140(12): 793-6, 2013 Dec.
Artigo em Francês | MEDLINE | ID: mdl-24315226

RESUMO

BACKGROUND: IgA pemphigus is a particular entity among autoimmune blistering intraepidermal diseases. IgA pemphigus is subdivided into two types: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis. PATIENTS AND METHODS: We report the case of an 82-year-old woman with intraepidermal neutrophilic IgA pemphigus associated with IgA gammopathy. The histopathological findings were unusual, with numerous large subcorneal pustules, a few pustules in the stratum spinosum, and basal IgA deposition. A favourable outcome was achieved with acitretin. DISCUSSION: This observation is significant in that it highlights the difficulty of classification of IgA pemphigus, which is currently based on clinical and histopathological findings. There is currently no therapeutic consensus attitude but simply a set of empirical data.


Assuntos
Acitretina/uso terapêutico , Imunoglobulina A/sangue , Cadeias kappa de Imunoglobulina/sangue , Ceratolíticos/uso terapêutico , Gamopatia Monoclonal de Significância Indeterminada/complicações , Pênfigo/tratamento farmacológico , Idoso de 80 Anos ou mais , Betametasona/uso terapêutico , Proteína C-Reativa/análise , Dapsona/uso terapêutico , Feminino , Humanos , Imunoglobulina A/análise , Infiltração de Neutrófilos , Pênfigo/complicações , Pênfigo/imunologia , Pênfigo/patologia , Recidiva , Pele/imunologia , Pele/patologia
20.
Br J Dermatol ; 168(3): 555-62, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23136927

RESUMO

BACKGROUND: Drug patch tests (PTs) can reproduce delayed hypersensitivity to drugs and entail a moderate re-exposure of patients to offending drugs. OBJECTIVES: To determine the value of PTs for identifying the responsible drug in severe cutaneous adverse drug reactions (SCARs) such as acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS) and Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). METHODS: In a multicentre study, PTs were conducted on patients referred for DRESS, AGEP or SJS/TEN within 1 year of their SCAR. All drugs administered in the 2 months prior to and the week following the onset of the SCAR were tested. RESULTS: Among the 134 patients included (48 male, 86 female; mean age 51·7 years), positive drug PTs were obtained for 24 different drugs. These included positive tests for 64% (46/72) of patients with DRESS, 58% (26/45) of those with AGEP and 24% (4/17) of those with SJS/TEN, with only one relapse of AGEP. The value of PTs depended on the type of drug and the type of SCAR (e.g. carbamazepine was positive in 11/13 DRESS cases but none of the five SJS/TEN cases). PTs were frequently positive for beta lactams (22 cases), pristinamycin (11 cases) and in DRESS with pump proton inhibitors (five cases), but were usually negative for allopurinol and salazopyrin. Of 18 patients with DRESS, eight had virus reactivation and positive PTs. In DRESS, multiple drug reactivity was frequent (18% of cases), with patients remaining sensitized many years later. CONCLUSIONS: PTs are useful and safe for identifying agents inducing SCAR.


Assuntos
Toxidermias/diagnóstico , Pustulose Exantematosa Aguda Generalizada/induzido quimicamente , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Toxidermias/etiologia , Interações Medicamentosas , Eosinofilia/induzido quimicamente , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes do Emplastro/efeitos adversos , Testes do Emplastro/métodos , Síndrome de Stevens-Johnson/induzido quimicamente , Síndrome de Stevens-Johnson/etiologia , Fatores de Tempo , Adulto Jovem
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