RESUMEN
Darier disease (DD) is an autosomal dominant acantholytic dermatosis with an estimated prevalence of 1 in 30 000-100 000. A localized form of DD was first described by Kreibich in 1906 and is thought to account for 10% of all cases. A number of clinical variants have been reported including: unilateral, linear, segmental or zosteriform DD. We present a case series of three patients with localized DD.
Asunto(s)
Enfermedad de Darier/genética , Enfermedad de Darier/patología , Mosaicismo , Adulto , Edad de Inicio , Anciano , Enfermedad de Darier/clasificación , Femenino , Humanos , Masculino , Persona de Mediana EdadAsunto(s)
Liquen Escleroso y Atrófico/etiología , Derivación Urinaria/efectos adversos , Administración Tópica , Anciano , Antiinflamatorios/uso terapéutico , Fluocinonida/uso terapéutico , Glucocorticoides , Humanos , Liquen Escleroso y Atrófico/tratamiento farmacológico , Masculino , Úlcera Cutánea/tratamiento farmacológico , Úlcera Cutánea/etiologíaRESUMEN
We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.
