RESUMEN
BACKGROUND: No firm data are available on the natural history of idiopathic thrombocytopenic purpura (ITP) or on mortality rates or frequency of major bleeding episodes associated with this condition. The disease is thought to have a relatively benign course, despite the frequent occurrence of very low platelet counts. This prevailing conception often guides therapeutic decisions. OBJECTIVE: To estimate the bleeding risk of ITP involving persistent low platelet counts (<30 x 10(9)/L) and its impact on prognosis. DESIGN: Age-adjusted bleeding risk was derived from a pooled analysis of ITP clinical series based on a systematic literature search. The risk estimate was incorporated into a Markov model to determine its impact on prognosis. RESULTS: Seventeen case series complied with inclusion criteria, including 1,817 patients with ITP. There were 49 cases of fatal hemorrhage over an estimated 1,258 to 3,023 patient-years at risk. The rate of fatal hemorrhage before age adjustment was estimated at between 0.0162 and 0.0389 cases per patient-year. Age-adjusted rates were 0.004, 0.012, and 0.130 cases per patient-year for age groups younger than 40, 40 to 60, and older than 60 years, respectively. Predicted 5-year mortality rates ranged from 2.2% for patients younger than 40 years to 47.8% for those older than 60 years. A 30-year-old woman remaining thrombocytopenic due to ITP was predicted to lose 20.4 years (14.9 quality-adjusted life years) of her potential life expectancy. At age 70, predicted loss was 9.4 years (5.0 quality-adjusted life years). CONCLUSIONS: Idiopathic thrombocytopenic purpura with persistent low platelet counts carries a grave prognosis. Therefore, an active therapeutic approach in the clinical management of affected patients should be considered. In view of the significant potential implications of the model results, we call for initiating a well-designed prospective inception cohort study of patients with ITP.
Asunto(s)
Hemorragia/etiología , Púrpura Trombocitopénica Idiopática/complicaciones , Adulto , Factores de Edad , Anciano , Hemorragia Cerebral/etiología , Simulación por Computador , Femenino , Hemorragia/sangre , Humanos , Masculino , Cadenas de Markov , Persona de Mediana Edad , Modelos Biológicos , Recuento de Plaquetas , Púrpura Trombocitopénica Idiopática/sangre , Reproducibilidad de los Resultados , Factores de Riesgo , Sensibilidad y Especificidad , Accidente Cerebrovascular/etiologíaAsunto(s)
Neoplasias de la Mama/patología , Carcinoma Ductal de Mama/secundario , Neoplasias Faciales/secundario , Melanoma/patología , Neoplasias Cutáneas/secundario , Adulto , Neoplasias Óseas/secundario , Carcinoma Ductal de Mama/patología , Diagnóstico Diferencial , Neoplasias Faciales/patología , Femenino , Humanos , Costillas/patología , Neoplasias Cutáneas/patologíaRESUMEN
BACKGROUND: Darier's disease is an acantholytic dyskeratotic genodermatosis with autosomal dominant inheritance. A predictive diagnostic marker for this disorder would be beneficial because of the relatively late onset and the large number of sporadic cases of the disease. The dermatoglyphic features of patients with Darier's disease were examined to determine whether they have a common pattern. METHODS: Ink prints of fingers and palms obtained from 11 patients of both sexes with sporadic and familial Darier's disease were analyzed and compared with those of normal subjects. RESULTS: No significant quantitative or qualitative differences were found between the dermatoglyphic features of our patients and those of a healthy population, except for punctate interruptions of the skin ridges that indicate pitting, a well-known manifestation of Darier's disease. CONCLUSIONS: These results refute the conclusions of a previous publication claiming that there is a common characteristic dermatoglyphic feature in patients with this dermatosis.
Asunto(s)
Enfermedad de Darier/diagnóstico , Dermatoglifia/clasificación , Adolescente , Adulto , Niño , Enfermedad de Darier/congénito , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND AND DESIGN: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. RESULTS: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. CONCLUSIONS: These findings indicate that there is an immunologic involvement in primary anetoderma.
Asunto(s)
Tejido Elástico/inmunología , Enfermedades de la Piel/inmunología , Adulto , Aglutininas/análisis , Anticuerpos Antinucleares/análisis , Atrofia , Autoanticuerpos/análisis , Enfermedades Autoinmunes/inmunología , Enfermedades del Tejido Conjuntivo/complicaciones , Enfermedades del Tejido Conjuntivo/inmunología , Enfermedades del Tejido Conjuntivo/patología , Crioglobulinas , Tejido Elástico/patología , Femenino , Técnica del Anticuerpo Fluorescente , Enfermedad de Graves/complicaciones , Hemólisis , Humanos , Hipergammaglobulinemia/inmunología , Inmunoglobulina G/análisis , Inhibidor de Coagulación del Lupus/análisis , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/complicaciones , Enfermedades de la Piel/complicaciones , Enfermedades de la Piel/patología , Tromboflebitis/complicaciones , Glándula Tiroides/inmunologíaRESUMEN
Although the underlying pathologic mechanisms of primary anetoderma have not yet been identified, data suggest the participation of an immunologic mechanism in some cases. In a woman with clinical and histopathologic features of primary anetoderma (Jadassohn-Pellizzari type) of 30 years' duration, laboratory investigation disclosed positive antinuclear factor, hypocomplementemia, hypergammaglobulinemia, granular deposits of immunoreactants along the dermoepidermal junction, and fibrillar deposits in the papillary dermis. In addition, she was found to have autoimmune hemolysis and circulating lupus anticoagulant associated with recurrent deep-vein thrombosis and a history of Graves' disease (starting 5 years after onset of primary anetoderma). To our knowledge, none of the latter three autoimmune conditions has been previously associated with primary anetoderma.
Asunto(s)
Enfermedades Autoinmunes , Enfermedades de la Piel , Anticuerpos Antinucleares/análisis , Atrofia , Autoanticuerpos/análisis , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/patología , Trastornos de la Coagulación Sanguínea/inmunología , Factores de Coagulación Sanguínea/análisis , Factores de Coagulación Sanguínea/inmunología , Tejido Elástico/patología , Femenino , Técnica del Anticuerpo Fluorescente , Humanos , Inmunoglobulina G/análisis , Inmunoglobulina M/análisis , Inhibidor de Coagulación del Lupus , Lupus Eritematoso Sistémico/inmunología , Persona de Mediana Edad , Fosfolípidos/inmunología , Enfermedades de la Piel/inmunología , Enfermedades de la Piel/patologíaRESUMEN
Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.