ABSTRACT
La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.
Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Cicatrix/drug therapy , Pemphigoid, Benign Mucous Membrane/complications , Stevens-Johnson Syndrome/complications , Conjunctivitis/drug therapy , Rituximab/administration & dosage , Immunologic Factors/administration & dosage , Chronic Disease , Cicatrix/etiology , Treatment Outcome , Conjunctivitis/etiologyABSTRACT
O penfigoide de membranas mucosas é entidade nosológica encarada como um fenótipo, que engloba várias dermatoses autoimunes com lesões bolhosas subepidérmicas, ocorrendo predominantemente nas membranas mucosas, com êxito cicatricial. O acometimento esofágico no penfigoide de membranas mucosas é raro e observado em pacientes com lesão disseminada. As alterações mais comuns são múltiplas membranas ou constrições esofagianas. No presente relato, os autores apresentam paciente com PMM sem lesões cutâneas e estenose esofágica grave, que entrou em remissão após uso de imunoglobulina venosa.
Mucous membrane pemphigoid (MMP) is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multiple esophageal membranes or strictures. In the present case, the authors report on a patient with MMP without any skin lesions and with severe esophageal strictures who went into remission following use of intravenous immunoglobulin.
Subject(s)
Aged , Female , Humans , Esophageal Stenosis/etiology , Pemphigoid, Benign Mucous Membrane/complications , Esophageal Stenosis/diagnosis , Esophageal Stenosis/drug therapy , Glucocorticoids/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Prednisone/therapeutic use , Severity of Illness IndexABSTRACT
El penfigoide cicatricial o de mucosas es una patología que afecta principalmente a la mucosa oral y ocular. Sólo en 25% de los pacientes afecta también la piel y en alrededor de 10% de los pacientes compromete a la mucosa faringo-laríngea, produciendo en los casos severos disnea que incluso puede requerir traqueostomía. En el tratamiento del penfigoide cicatricial habitualmente se utilizan corticoides e inmunosupresores. En este artículo presentamos un caso de una mujer de 69 años con diagnóstico de penfigoide cicatricial que se manifiesta con disfagia y se comprueba con nasofibroscopía una estenosis supraglótica. Se discute el diagnóstico y manejo de esta patología poco habitual.
Cicatricial or mucous membranes pemphigoid is a rare pathology that affects mainly the ocular and oral mucosae. In only 25% of patients the skin is affected, and in 10%, the pharyngolaryngeal mucosa, resulting in serious cases in dyspnea that could even require a tracheostomy. Treatment is usually based on corticoids and immunosupressors. In this article we present the case of a 69 year old woman diagnosed with cicatricial pemphigoid, that presented dysphagia. A nasoendoscopy revealed supraglottic stenosis. Diagnosis and treatment of this uncommon disease are discussed.
Subject(s)
Humans , Female , Aged , Laryngostenosis/etiology , Pemphigoid, Benign Mucous Membrane/complications , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/therapy , Dyspnea/etiology , Deglutition Disorders/etiology , Oral Ulcer/etiologyABSTRACT
Penfigóide Cicatricial (Penfigóide Cicatricial de Membrana Mucosa) é uma doença auto-imune inflamatória crônica caracterizada pela presença de bolhas subepiteliais em membranas mucosas e, ocasionalmente na pele. Pode haver acometimento oral, nasal, faríngeo, laríngeo, ocular, esofágico, anogenital e de pele, predominantemente em pacientes na quinta e sexta décadas de vida. O tratamento preconizado inclui corticóides sistêmicos e agentes imunossupressores. Descrevemos neste trabalho dois casos com a doença em atividade, sendo que um evoluiu com complicação séptica pelo uso de imunossupressor e outro que apresentou estenose supraglótica exigindo traqueotomia.
Cicatricial pemphygoid (mucous membrane cicatricial pemphygoid) is a chronic autoimmune inflammatory disease characterized by subepithelial bubbles in mucous membranes and, occasionally on the skin. It may affect the mouth, the nose, pharynx, larynx, the eyes, esophagus, anus, genitals and skin; especially affecting patients between fifty and sixty years of life. Treatment includes systemic steroids and immunosuppressive agents. In the present paper we describe two cases with the active disease, and one of them had sepsis because of using immunosuppressive agents and another that presented supraglottic stenosis requiring tracheostomy.
Subject(s)
Aged , Humans , Male , Middle Aged , Mouth Diseases/etiology , Pemphigoid, Benign Mucous Membrane/complications , Mouth Diseases/diagnosis , Mouth Diseases/drug therapy , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapyABSTRACT
Se presenta una paciente de sexo femenino, con penfigoide benigno de las mucosas de nueve años de evolución, con disfagia progresiva alta. El estudio endoscópico reveló la presencia de membranas en el sófago, dispuestas a modo de tabiques que generaban retracción a nivel de su inserción. La paciente además presentaba gingivitis descamativa en la mucosa bucal, que se acompañaba con sinequias bilaterales de la conjuntiva ocular a nivel del ángulo externo. La histopatología comprobó el diagnóstico de penfigoide mucoso benigno; la inmunofluorescencia directa en piel, mostró depósito de IgA en zona dermoepidérmica con aspecto tubular. La disfagia desapareció con el debridamiento de la zona afectada, mediante la endoscopía