RESUMEN
Mucous membrane pemphigoid (MMP) is often accompanied with a chronic cicatrizing conjunctivitis that may eventually lead to loss of vision. The study is intended to investigate the current scientific knowledge on the diagnosis of and therapy for MMP involving the eye. Previous studies published before December 2004 have been systematically reviewed for their level of evidence. Consequently, recommendations for patient management are provided.
Asunto(s)
Conjuntivitis/diagnóstico , Conjuntivitis/terapia , Queratitis/diagnóstico , Queratitis/terapia , Penfigoide Benigno de la Membrana Mucosa/diagnóstico , Penfigoide Benigno de la Membrana Mucosa/terapia , Enfermedad Crónica , Conjuntivitis/complicaciones , Progresión de la Enfermedad , Humanos , Queratitis/complicaciones , Penfigoide Benigno de la Membrana Mucosa/complicaciones , Guías de Práctica Clínica como Asunto , Pautas de la Práctica en Medicina , Pronóstico , Resultado del Tratamiento , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/etiología , Trastornos de la Visión/terapiaRESUMEN
The clinical features of a 76-year-old man with histiocytosis X of the Letterer-Siwe type, with extensive skin involvement, are described. The patient's lesions responded dramatically to PUVA-photochemotherapy. Light microscopic, immunocytochemical and immunoelectron microscopic findings, before and after PUVA, are reported. Birbeck granules in phagolysosomes of dermal macrophages indicated uptake of destroyed HX cells. Residual HX cells in the skin and HX cells in a recurring lesion expressed the same membrane antigens as in the primary lesions.