Penfigoide cicatricial esofágico / Oesophageal cicatricial pemphigoid

No disponible

Characterization of Corneal Involvement in Eyes With Mucous Membrane Pemphigoid by In Vivo Confocal Microscopy.

PURPOSE: To describe the morphological features of the corneal epithelial layers, subbasal nerve plexus, stroma, and endothelium in patients with mucous membrane pemphigoid (MMP) as shown by in vivo confocal microscopy (IVCM). METHODS: Central corneal images were captured from 10 healthy age-matched control eyes and 30 eyes with clinically diagnosed MMP by in vivo laser scanning confocal microscopy (HRT III RCM). Morphological changes of the corneal epithelial layers, stroma, and endothelium, characteristics of corneal nerves, and presence of inflammatory dendritic cells (DCs) were evaluated. RESULTS: Images obtained by IVCM from 40 eyes were analyzed. The eyes with MMP were divided into 2 groups based on clinical staging: 16 eyes with end-stage MMP and 14 eyes with non-end-stage MMP. Compared with controls, IVCM in eyes with end-stage MMP displayed severe conjunctivalization and neovascularization of the cornea, with otherwise limited identifiable cellular or structural elements. Those with non-end-stage MMP showed metaplasia of the corneal epithelial layers, presence of hyperreflective cells similar to conjunctival cells, intraepithelial defects, fibrosis of anterior stroma, and hyperreflective endothelial deposits. Images of the subbasal nerve plexus demonstrate significant reduction in density (1251.3 ± 806.9 µm/frame vs. 2688.8 ± 607.33 µm/frame, P < 0.001), increased tortuosity (2.76 ± 0.6 vs. 2.3 ± 0.42, not significant), decreased reflectivity (2.73 ± 0.4 vs. 3.46 ± 0.52, P < 0.01), and increased density of DCs (115 ± 88 cells/mm vs. 43.9 ± 28.14 cells/mm, P < 0.05) in MMP-affected eyes compared with controls. CONCLUSIONS: IVCM reveals profound and variable microstructural changes in the corneas of patients with MMP compared with normal controls. Our study demonstrated decreased corneal nerve density and elevated DC density in eyes with non-end-stage MMP compared with normal controls. Frequent scarring, conjunctivalization, and neovascularization observed in eyes with end-stage MMP preclude recognition by IVCM of the morphologic architecture of the corneal layers. Our findings suggest implications for using IVCM to evaluate and monitor patients with MMP.

Seguimiento multidisciplinario en el control del penfigoide de membranas mucosas / Multidisciplinary follow-up in mucous membranes pemphigoid control

El término penfigoide de membranas mucosas designa un grupo de enfermedades vesículo-bulosas autoinmunes crónicas, que afectan predominantemente mucosas. El presente artículo describe dos casos clínicos de penfigoide de membranas mucosas con compromiso gingival y tuvo como objetivo resaltar la importancia de un correcto diagnóstico de la lesión y discutir la conducta terapéutica. Fueron realizados diversos exámenes complementares como, examen de Nikolsky, biopsia incisional, investigación de células LE y de anticuerpos antinucleares, además de la evaluación de médicos especializados en dermatología y oftalmología. Fue fundamental la substitución de dentífricos, la orientación nutricional y psicológica, el cuidado con la higiene, además del uso de corticoides tópicos. Hubo periodos de remisión y exacerbación del cuadro clínico durante el seguimiento, fue necesario el ajuste en la terapéutica y refuerzo en los cuidados con la higiene bucal. Es muy importante la interacción multidisciplinaria en la atención de estos casos para el control del tratamiento y seguimiento, además de reforzar todas las orientaciones y cuidados en lo que se refiere a la salud bucal y cautela en el uso de corticoides(AU)

The term Mucous Membrane Pemphigoid includes a group of chronic and autoimmune vesiculobullous diseases involving mainly the mucosa. The aim of present paper is to describe two clinical cases of Mucous Membrane Pemphigoid with gingival involvement to arrive to a proper diagnosis of the lesion and also to discuss the therapeutic behavior. Different complementary examinations were conducted including Nikolsky test, incisional biopsy, the LE cells study and of antinuclear antibodies, as well as the evaluation of dermatologists and ophthalmologists. It was necessary the replacement of toothpastes, the nutritional and psychological guiding, the hygiene care and the use of topic corticoids. There were periods of remission and exacerbation of clinical picture during follow-up being necessary the therapeutics fitting and the oral hygiene care reinforcement. It is important the multidisciplinary interaction in the care of these cases for treatment and follow-up control, as well as to reinforce all orientations and cares related to oral hygiene and the caution in the use of corticoids(AU)

Barium studies in esophageal cicatricial pemphigoid.

BACKGROUND: Cicatricial pemphigoid (CP) (benign mucous membrane pemphigoid) is a rare, blistering disease of skin and mucous membrane. The disease rarely extends to involve the esophagus, and there are only a few cases reported in the radiological literature. The aims of this study were to document the frequency of esophageal involvement and to describe the findings on upper GI barium studies. METHODS: A total of 197 patients with CP were seen at our institution from 1981 to 1991. The clinical and radiological findings of these patients were reviewed and compared with findings reported in the literature. RESULTS: Esophageal involvement was documented in seven patients. Cervical esophageal webs were found in five of the seven patients. Two patients had single esophageal webs while three had multiple webs. Frank strictures of the esophagus were also seen in five patients. These were most common in the cervical esophagus, but strictures were also found in the mid and lower esophagus. Two of the strictures resulted in significant dysphagia and required multiple endoscopic dilatations. One of the dilatations was complicated by mucosal injury, and follow-up barium examination showed dissection of the esophageal mucosa from the cervical esophagus to the esophagogastric junction. One patient demonstrated intramural pseudodiverticulosis in the cervical esophagus. Functional disturbances demonstrated on barium studies included tracheal aspiration in two patients and nasopharyngeal reflux in three. CONCLUSIONS: CP involves the esophagus in approximately 5% of cases. The hypopharynx and cervical esophagus are most commonly involved, but any portion of the esophagus may be involved, and multiple levels of involvement may be seen. Cervical esophageal webs, often multiple or complex, are the most common appearance on barium studies, but frank strictures are also found. Secondary manifestations of esophageal involvement include nasopharyngeal reflux, tracheal aspiration, and intramural pseudodiverticulosis.

Oesophageal involvement in benign mucous membrane pemphigoid.

Benign mucous membrane pemphigoid is a relatively rare disease characterised by the presence of bullous lesions and erosions of the mucous membranes leading to scarring. Oral and conjunctival mucosae are most frequently affected. Occasionally, the oesophagus is involved and two radiological patterns, webs and smooth strictures, are seen.