RESUMEN
A 13-year-old child is described who presented with generalized pemphigus vulgaris associated with extraordinarily high titers of circulating autoantibodies against the pemphigus antigen. Because of the lack of response to treatment with reasonably high doses of oral corticosteroids, as well as the very high titer of circulating autoantibodies observed, this patient was treated with intravenous corticosteroid pulse therapy followed by plasmapheresis and then by combination immunosuppressive therapy (prednisone and azathioprine). A rapid clinical response was induced, correlating with reduction and subsequent elimination of the circulating pemphigus autoantibodies. Using such combination therapy, a remission of 12 months was achieved, and prednisone therapy was completely, albeit temporarily, tapered and then discontinued. Subsequent disease flare was then easily controlled with a short course of low-dose oral corticosteroid therapy.
Asunto(s)
Azatioprina/uso terapéutico , Metilprednisolona/uso terapéutico , Pénfigo/terapia , Plasmaféresis , Prednisona/uso terapéutico , Adolescente , Complejo Antígeno-Anticuerpo/análisis , Autoanticuerpos/análisis , Azatioprina/administración & dosificación , Terapia Combinada , Quimioterapia Combinada , Femenino , Técnica del Anticuerpo Fluorescente , Humanos , Inyecciones Intravenosas , Metilprednisolona/administración & dosificación , Pénfigo/tratamiento farmacológico , Pénfigo/inmunología , Pénfigo/patología , Prednisona/administración & dosificaciónRESUMEN
Three members of a family with numerous ectodermal abnormalities are described. These anomalies primarily include patchy alopecia beginning in childhood, premature cataracts, widespread keratosis pilaris, and psoriasis. The alopecia and premature cataracts appear to follow an autosomal dominant inheritance pattern with incomplete penetrance and appear to be linked. Psoriasis also occurs in several members of this family and probably represents a separate but possibly related genodermatosis. This kindred has features of both keratosis follicularis spinulosa decalvans and ichthyosis follicularis, and the disorder seems to fit into the group of follicular hyperkeratosis disorders.
Asunto(s)
Alopecia/genética , Catarata/genética , Queratosis/genética , Psoriasis/genética , Adulto , Femenino , Genes Dominantes , Ligamiento Genético , Humanos , LinajeRESUMEN
A patient with erythema gyratum repens in whom a bronchogenic carcinoma was found is described. Erythema gyratum repens is a cutaneous eruption with a unique morphology resembling a wood grain pattern. Its presence is almost always associated with serious systemic pathology, usually neoplastic, and thus should be considered a cutaneous marker of internal malignancy.