Asunto(s)
Inhibidores de la Dipeptidil-Peptidasa IV , Penfigoide Ampolloso , Enfermedades Cutáneas Vesiculoampollosas , Humanos , Penfigoide Ampolloso/inducido químicamente , Penfigoide Ampolloso/tratamiento farmacológico , Inhibidores de la Dipeptidil-Peptidasa IV/efectos adversos , Técnica del Anticuerpo Fluorescente Directa , Colágenos no Fibrilares , Hipoglucemiantes , Coloración y Etiquetado , Dipeptidil-Peptidasas y Tripeptidil-Peptidasas , Autoanticuerpos , AutoantígenosRESUMEN
BACKGROUND: There is controversy over late and long-lasting reactions to gold sodium thiosulfate (GST). OBJECTIVES: To study the GST patch-test reaction by observing the application site after 1 month, and to clarify the relevance of GST sensitization by piercings and dental metals. PATIENTS: A retrospective analysis was performed on 746 patients (143 male; 603 female) who were patch tested using GST of the TRUE Test. We conducted a questionnaire on the presence of piercings or dental metals in these patients. RESULTS: The GST positive rate was 27.9% at day (D)3 and/or D7 and 40.3% up to the 1-month reading. The positive rate was significantly higher in female patients and increased with age. Sixty-two percent of cases with a positive reaction at D7 continued to show a positive reaction after 1 month. Eleven percent of cases with a negative reaction at D3 and D7 showed a late reaction. Both piercings and dental metals were related to gold sensitization. CONCLUSIONS: The GST of the TRUE Test had a high positive and low false-negative rate. The 1-month reading after the patch test was important for identifying late reactions. Piercing history and dental metal were associated with gold sensitization.
Asunto(s)
Antihipertensivos/efectos adversos , Complemento C3/metabolismo , Eosinófilos/patología , Neutrófilos/patología , Enfermedades Cutáneas Vesiculoampollosas/inducido químicamente , Enfermedades Cutáneas Vesiculoampollosas/patología , Anciano de 80 o más Años , Membrana Basal/metabolismo , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Blastic plasmacytoid dendritic cell (pDC) neoplasm (BPDCN) is a relatively rare hematological malignancy with significantly complex clinicopathological features that are still unclear. This study aimed to analyze the clinicopathological data of BPDCN and evaluate immunohistochemical detection of minimal bone marrow (BM) involvement. In this study, we examined skin and BM lesions from 6 patients with BPDCN. Neoplastic cells tested positive for CD303 (polyclonal, 100%; monoclonal, 40%) in the skin lesions and for CD303 (polyclonal, 100%; monoclonal, 67%) in the BM clots. Although immunostaining of CD4, CD56, CD123, CD303, and TCLl detected minimal BM involvement in 3 patients, morphological identification was challenging in the BM clots stained with hematoxylin-eosin. In conclusion, our results demonstrate the significance of observing BM smears to detect neoplastic cells and that immunohistochemical examination, including CD303 antibodies, is useful to detect minimal BM involvement. This study is the first to report the expression of thymic stromal lymphopoietin (TSLP) and its receptor in BPDCN cells. Therefore, the TSLP/TSLP receptor axis may be associated with the proliferation of BPDCN, and consequently, the survival of patients.
Asunto(s)
Células de la Médula Ósea , Células Dendríticas , Lectinas Tipo C/metabolismo , Glicoproteínas de Membrana/metabolismo , Proteínas de Neoplasias/metabolismo , Células Plasmáticas , Receptores Inmunológicos/metabolismo , Adulto , Anciano , Anciano de 80 o más Años , Biopsia con Aguja , Células de la Médula Ósea/metabolismo , Células de la Médula Ósea/patología , Células Dendríticas/metabolismo , Células Dendríticas/patología , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patología , Humanos , Inmunohistoquímica , Masculino , Células Plasmáticas/metabolismo , Células Plasmáticas/patologíaRESUMEN
A 54-year-old Japanese woman had repetitive superficial skin peeling and ensuing erythematous changes in the sites since infancy. Her parents had a consanguineous marriage, and she was the only individual affected in her family tree. The erythematous changes seemed to worsen in the summer. Histologically, hyperkeratosis and splitting of the epidermis within the stratum corneum was noted, and electron microscopy revealed shedding of corneal cells in the horny layer and normal-looking corneodesmosomes. Gene analysis revealed a homozygous missense mutation at c.1358G>A in CDSN. Electron microscopic examination of the length and number of corneodesmosomes revealed statistically significant shortness and sparsity in the affected individual (mean ± SD 386.2 ± 149.5 nm) compared with that of an age- and site-matched control (406.6 ± 182.3 nm). We speculate that this size shrinkage of corneodesmosomes might be the result of a missense mutation of CDSN and that this could be one of the factors contributing to the pathological process of skin peeling.
RESUMEN
A 38-year-old woman was suffering from irregular headaches and sleepiness. She had used soap containing Glupearl 19S (hydrolyzed wheat proteins) every day for approximately one year and had experienced an episode of rash eruption on her face seven months ago. Wheat-specific IgE antibodies were detected in her serum. A Western blot analysis revealed a high titer of IgE antibodies against Glupearl 19S and wheat proteins. The patient was sensitive to these compounds in a skin prick test. After avoiding eating wheat, her headaches and sleepiness disappeared. A hidden food allergy is a possible cause of these symptoms.
Asunto(s)
Trastornos de Somnolencia Excesiva/etiología , Trastornos de Cefalalgia/etiología , Proteínas de Plantas/efectos adversos , Jabones/efectos adversos , Hipersensibilidad al Trigo/complicaciones , Adulto , Antipruriginosos/uso terapéutico , Conjuntivitis Alérgica/tratamiento farmacológico , Conjuntivitis Alérgica/etiología , Femenino , Antagonistas de los Receptores Histamínicos/uso terapéutico , Humanos , Hidrólisis , Inmunoglobulina E/sangre , Inmunoglobulina E/inmunología , Proteínas de Plantas/inmunología , Hipersensibilidad al Trigo/diagnóstico , Hipersensibilidad al Trigo/dietoterapiaAsunto(s)
Alérgenos/efectos adversos , Alérgenos/inmunología , Anafilaxia/etiología , Glicoproteínas/efectos adversos , Glicoproteínas/inmunología , Proteínas de Insectos/efectos adversos , Proteínas de Insectos/inmunología , Adulto , Anafilaxia/inmunología , Animales , Femenino , Humanos , Inmunoglobulina E/sangre , Japón , Proteínas de Unión al ARN , Pruebas CutáneasAsunto(s)
Granuloma Piogénico/cirugía , Preescolar , Cara , Femenino , Granuloma Piogénico/patología , Humanos , Ligadura , Resultado del TratamientoAsunto(s)
Tumores del Estroma Gastrointestinal/complicaciones , Síndromes Paraneoplásicos/etiología , Pénfigo/etiología , Corticoesteroides/uso terapéutico , Autoanticuerpos/sangre , Procedimientos Quirúrgicos del Sistema Digestivo , Disnea/etiología , Resultado Fatal , Tumores del Estroma Gastrointestinal/diagnóstico por imagen , Tumores del Estroma Gastrointestinal/cirugía , Humanos , Masculino , Persona de Mediana Edad , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/patología , Síndromes Paraneoplásicos/terapia , Pénfigo/inmunología , Pénfigo/patología , Pénfigo/terapia , Prednisona/uso terapéutico , Piel/patología , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. Thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV DNA (PCR) 8 x 10(4). EBV-encoded small nuclear RNA (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g DNA in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.
Asunto(s)
Infecciones por Virus de Epstein-Barr/diagnóstico , Dermatosis Facial/diagnóstico , Hidroa Vacciniforme/diagnóstico , Dermatosis del Cuero Cabelludo/diagnóstico , Preescolar , Enfermedad Crónica , ADN Viral/genética , Diagnóstico Diferencial , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/patología , Dermatosis Facial/complicaciones , Dermatosis Facial/patología , Antebrazo/patología , Herpesvirus Humano 4/aislamiento & purificación , Humanos , Hidroa Vacciniforme/complicaciones , Hidroa Vacciniforme/patología , Hibridación in Situ , Masculino , Reacción en Cadena de la Polimerasa , Dermatosis del Cuero Cabelludo/complicaciones , Dermatosis del Cuero Cabelludo/patología , Tórax/patologíaRESUMEN
A 59-year-old Japanese man with pyoderma gangrenosum occurring at the unusual location of the ear lobe is herein reported. The patient was not associated with any other systemic diseases and had suffered from chilblains at the same site for ten years before the ulcer appeared. The ulcer followed the development of a purpuric exudative lesion and had neither an undermined nor a surpiginous border in the early lesion. It gradually increased in size after various conservative treatments, recurred within a month after being excised and became aggravated after the administration of potassium iodide. Repeated histopathology of the ulcer revealed a mixed inflammatory cell infiltrate with abscesses and an extravasation of red blood cells in the whole dermis, without showing leukocytoclastic vasculitis. A culture of the excised tissue yielded no growth. Laboratory tests were not specific and c-ANCA was also negative. The ulcer of the ear did dramatically respond to systemic predonisolone of 40 mg/day. The auricular and periauricular area are quite rare anatomical sites of this disease and the difference between pyoderma gangrenosum and cutaneous Wegener's granulomatosis is also discussed.