Characteristics Upon Presentation of Ocular Mucous Membrane Pemphigoid Patients in Puerto Rico.

OBJECTIVE: To describe the characteristics upon presentation of a cohort of Hispanic patients living in Puerto Rico with ocular mucous membrane pemphigoid (MMP). METHODS: Retrospective chart review of subjects with ocular MMP at one academic institution and one private practice. Patients with clinical evidence of ocular MMP, along with a positive mucous membrane biopsy revealing linear antibody or C3 deposition in the basement membrane zone, or with a positive indirect immunofluorescence assay were included. Descriptive statistical analysis was performed. RESULTS: Eight patients with ocular mucous membrane pemphigoid were identified. The median age upon presentation was 60.5 years; however, 2 patients were in their 4th decade and one in the 5th decade of life. Females constituted 62.5% of the cohort. All patients presented with stage III ocular MMP in at least one eye and 50% had history of trichiasis. Seven out of eight patients (87.5%) had extraocular symptoms for a median duration of 36 months (range 2-144 months). The most common site of extraocular involvement was the oropharynx, present in 87.5% of patients. CONCLUSION: Our results suggest that in Puerto Rico ocular MMP most commonly presents in the seventh decade of life. The presence of symblepharon, trichiasis or oropharyngeal mucosal disease should prompt further evaluation and consideration for immunopathological tissue analysis and an IIF assay.

Penfigoide benigno de las mucosas: presentación de un caso / Bening pemphigoid of the mucous membranes: report of a clinical case

Fundamento: el penfigoide benigno de las mucosas es una enfermedad que afecta principalmente a la mucosa oral y ocular. El daño a la mucosa bucal es observado en la mayoría de los pacientesy cuando se presenta en la gingiva, produce un cuadro parecido al de una gingivitis descamativa. Objetivo: relatar un caso de penfigoide benigno de las mucosas donde el paciente presenta las lesiones solo en boca. Caso clínico: varón de 30 años, fumador, se presentó con diversas lesiones bucales de naturaleza vesiculoampollar, sangramiento espontáneo y provocado e intenso enrojecimiento de la encía. Al examen físico se observó un estado bucal desfavorable, caries, placa dentobacteriana, sarro, obturaciones caídas, recesiones gingivales generalizadas. No existió toma del estado general y los estudios analíticos dentro de límites normales. Se descartó lesiones oftalmológicas. Se discutió el diagnóstico y manejo de esta enfermedad poco habitual en hombres jóvenes. Se utilizó la prednisonapor vía oral, se dio el alta de los servicios dentales y se mantuvo una óptima higiene bucal por parte del paciente y vigilancia estricta del periodoncista. El penfigoide es una enfermedad grave y de larga evolución. Conclusiones: El paciente está en tratamiento, con una evolución satisfactoria.

Clinical and demographic overlaps among immunologically mediated oral diseases: a challenge for clinicians.

This study sought to assess and compare retrospective demographic and clinical data of oral lesions of lichen planus, pemphigus vulgaris, and mucous membrane pemphigoid from the records of the Department of Oral Pathology and Surgery, School of Dentistry, Universidade Federal de Minas Gerais, Brazil, covering a period of 55 years. Out of 25,435 specimens, these immunologically-mediated diseases accounted for 301 (1.18%) cases, of which 250 (0.98%) were lichen planus, 27 (0.11%) were pemphigus vulgaris, and 24 (0.09%) were mucous membrane pemphigoid. Lichen planus presented mainly as white asymptomatic plaques on buccal mucosa. Pemphigus vulgaris was usually characterized by multiple symptomatic erithematous ulcers on the buccal mucosa. Painful ulcers and/or blisters on the gingiva were the most common presentation for mucous membrane pemphigoid. Desquamative gingivitis was noted for all 3 diseases, but mainly for mucous membrane pemphigoid. Overall, lesions were more frequent in white women >50 years. Oral manifestations of immunologically-mediated diseases are relatively rare, and the correct diagnosis can be a challenge for dentists as the lesions often share similar clinical and demographic features.

Reflectance confocal microscopy as a new tool in the in vivo evaluation of desquamative gingivitis: patterns in mucous membrane pemphigoid, pemphigus vulgaris and oral lichen planus.

BACKGROUND: Desquamative gingivitis refers to a clinical manifestation associated with several mucocutaneous disorders. The most common are mucous membrane pemphigoid, pemphigus vulgaris and lichen planus. Their specific diagnosis is better established by histopathological and immunofluorescence evaluation. OBJECTIVES: To examine cases of desquamative gingivitis using reflectance confocal microscopy (RCM) and compare the findings with those of normal gingiva. To compare RCM findings in desquamative gingivitis with conventional histopathology of the biopsied lesions, in order to establish criteria for this noninvasive diagnostic technique. METHODS: A total of 25 cases of suspected mucous membrane pemphigoid, pemphigus vulgaris and lichen planus were included. RCM was performed on the gingiva of a healthy person and on gingival lesions. All lesions were biopsied in order to perform a RCM-histopathological correlation. RESULTS: Reflectance confocal microscopy examination of the gingival lesions suspected to be mucous membrane pemphigoid revealed a separation at the level of the dermal-epidermal junction, filled with small, bright structures interpreted as blood cells. Histopathological and immunofluorescence findings confirmed the diagnosis. For pemphigus vulgaris, RCM features were intraepithelial clefts with round, detached cells interpreted as acantholytic keratinocytes, similar to the histopathological features. Hyperkeratosis and spongiosis associated with infiltration of inflammatory cells, seen as small, bright cells intermingling with the honeycomb keratinocyte epithelial structure, were seen in lichen planus. Mildly bright, round structures interpreted as necrotic keratinocytes and mildly bright, stellate structures, interpreted as melanophages, were also seen in the dermis. These features were present on histopathology, confirming the diagnosis of lichen planus. CONCLUSION: We propose that RCM is a useful tool to help distinguish between the three most common causes of desquamative gingivitis.

[Systemic treatment of ocular cicatricial pemphigoid]. / Tratamiento sistémico del penfigoide cicatrizal ocular.

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppressive drugs. Four patients combined two immunosuppressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.

Tratamiento sistémico del penfigoide cicatrizal ocular / Systemic treatment of ocular cicatricial pemphigoid

El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ± 14 años, con un retraso de 7.5 ± 10 años. Sesenta se siguieron en nuestro servicio por 19 ± 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.

An epidemiological study of immune-mediated skin diseases affecting the oral cavity.

BACKGROUND: Immune-mediated skin diseases encompass a variety of pathologies that present in different forms in the body. OBJECTIVE: The objective of this study was to establish the prevalence of the principal immune-mediated skin diseases affecting the oral cavity. METHODS: A total of 10,292 histopathology reports stored in the archives of the Anatomical Pathology Laboratory, Department of Oral Pathology, Federal University of Rio Grande do Norte, covering the period from 1988 to 2009, were evaluated. For the cases diagnosed with some type of disease relevant to the study, clinical data such as the gender, age and ethnicity of the patient, the anatomical site of the disease and its symptomatology were collected. RESULTS: Of all the cases registered at the above-mentioned service, 82 (0.8%) corresponded to immune-mediated skin diseases with symptoms affecting the oral cavity. The diseases found in this study were: oral lichen planus, pemphigus vulgaris and benign mucous membrane pemphigoid. Oral lichen planus was the most common lesion, comprising 68.05% of the cases analyzed. Of these cases, 64.3% were women and the cheek mucosa was the anatomical site most commonly affected (46.8%). CONCLUSION: Immune-mediated skin diseases affecting the oral cavity continue to be rare, the prevalence found in this study being similar to that reported for the majority of regions worldwide. Nevertheless, early diagnosis is indispensable in the treatment of these diseases, bearing in mind that systemic involvement is possible in these patients.

Estudo epidemiológico das doenças dermatológicas imunologicamente mediadas na cavidade oral / An epidemiological study of immune-mediated skin diseases affecting the oral cavity

FUNDAMENTO: As doenças dermatológicas imunologicamente mediadas compõem diversas patologias que apresentam formas variadas de manifestação no organismo. OBJETIVO: Foi proposição desta pesquisa, estabelecer a prevalência das principais doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral. MÉTODOS: Foram avaliados laudos histopatológicos de 10.292 casos arquivados no Serviço de Anatomia Patológica da Disciplina de Patologia Oral da Universidade Federal do Rio Grande do Norte, no período de 1988 a 2009. Dos casos diagnosticados como algum tipo de doença em estudo, coletaram-se dados clínicos como sexo, idade, raça, sítio anatômico e sintomatologia das doenças. RESULTADOS: Do total de casos registrados, no serviço supracitado, 82 (0,8 por cento) corresponderam a doenças dermato lógicas imunologicamente mediadas com manifestação na cavidade oral. As doenças encontradas neste estudo foram: líquen plano oral, pênfigo vulgar e penfigoide benigno das membranas mucosas, sendo o líquen plano oral a lesão mais prevalente, representando 68,05 por cento dos casos analisados, dos quais 64,3 por cento apresentavam-se em mu lheres, sendo a mucosa jugal o sítio anatômico mais acometido (46,8 por cento). CONCLUSÃO: A ocorrência de doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral ainda é um fato incomum, semelhante ao observado na maioria das regiões mundiais. No entanto, a busca pelo diagnóstico precoce é um requisito essencial para a condução do tratamento dessas doenças, tendo em vista o possível comprometimento sistêmico do organismo nos pacientes.

BACKGROUND: Immune-mediated skin diseases encompass a variety of pathologies that present in different forms in the body. OBJECTIVE: The objective of this study was to establish the prevalence of the principal immune-mediated skin diseases affecting the oral cavity. METHODS: A total of 10,292 histopathology reports stored in the archives of the Anatomical Pathology Laboratory, Department of Oral Pathology, Federal University of Rio Grande do Norte, covering the period from 1988 to 2009, were evaluated. For the cases diagnosed with some type of disease relevant to the study, clinical data such as the gender, age and ethnicity of the patient, the anatomical site of the disease and its symptomatology were collected. RESULTS: Of all the cases registered at the above-mentioned service, 82 (0.8 percent) corresponded to immune-media ted skin diseases with symptoms affecting the oral cavity. The diseases found in this study were: oral lichen planus, pemphigus vulgaris and benign mucous membrane pemphigoid. Oral lichen planus was the most common lesion, comprising 68.05 percent of the cases analyzed. Of these cases, 64.3 percent were women and the cheek mucosa was the anatomical site most commonly affected (46.8 percent). CONCLUSION: Immune-mediated skin diseases affecting the oral cavity continue to be rare, the prevalence found in this study being similar to that reported for the majority of regions worldwide. Nevertheless, early diagnosis is indispensable in the treatment of these diseases, bearing in mind that systemic involvement is possible in these patients.

Penfigoide benigno de las mucosas: reporte de caso clínico / Benign pemphigoid of the oral mucosa: report of a clinical case

El penfigoide benigno de las mucosas es una enfermedad autoinmune ampollar. En un 80 por ciento de los casos está afectada la mucosa oral, de ahí la importancia que dicha enfermedad la conozca el odontólogo general y el especialista. El siguiente caso muestra el compromiso de la mucosa gingival en una paciente de 12 años de edad.

Childhood oral mucous membrane pemphigoid presenting as desquamative gingivitis in a 4-year-old girl.

Mucous membrane pemphigoid is a chronic inflammatory, bullous subepithelial auto-immune disease, with predominant involvement of the mucosal surfaces. Oral mucous membrane pemphigoid occurring in childhood is extremely rare. We describe a new case of this entity occurring in a 4-year-old girl who presented with desquamative gingivitis. Diagnosis was based on clinical presentation, histopathology, immunofluorescence and immunoblotting examinations. Treatment and 2-year follow-up are described.

Cicatricial pemphigoid: report of five cases.

Cicatricial pemphigoid is a chronic, systemic, autoimmune disease characterized by progressive bullous skin and mucous membrane lesions that tend toward scarring and involution. Manifestations of cicatricial pemphigoid include oral mucosal bullous lesions in 85 to 90% of patients, ocular mucosal lesions in 66%, nasal mucosal lesions in 15 to 23%, and laryngeal involvement in 8 to 21%. We report five cases of cicatricial pemphigoid in which all patients had ENT manifestations--specifically, oral and nasal mucosal involvement. Three of these patients also had laryngeal lesions; one of the three had a large laryngeal ulceration and bullae that caused a laryngeal stenosis and necessitated a tracheostomy. In addition to the five case reports, we also review the literature and discuss the pathogenesis, diagnosis, and treatment of this uncommon disease.

Analysis of desquamative gingivitis using direct immnuofluorescence in conjunction with histology

Chronic desquamative gengivitis (DG) is a commom condition often first recognized by periodontists. DG is characterized clinically by erythema with epithelial desquamation, ulceration, and/or the presence of vesiculobullous lesions of the free and attached gingiva. However, DG is actually a clinical manifestation of several different disease processes, partucularyly lichen planus and benign mucous membrane pemphigoid. Correct diagnosis in DG is critical since proper treatment anf follow-up will depend on which disease is involved. However, the diseases that cause DG frequently present diagnostic problems because their lesions often resemble each other clinically and routine histological examination sometimes canoot differentiate between them. Thus, immunohistology, particularly immunofluorescence, is increasingly being used with routine histology to more accurately diagnose DG diseases. This article reviews our experience over the past 10 years in the diagnosis of 72 cases of DG using direct immunofluorescent (DIF) in conjunction with histology and clinical evaluation. Of the 72 DG cases in this study, 30 cases were diagnosed as erosive lichen planus orlichen-oid mucositis; 29 cases were dianosed as benign mucous membrane pemphigoid; 2 cases each of linear IgA disease and pemphigus vulgaris were diagnosed; there was 1 case of bullous pemphegoid; and 1 suspected case of paraneoplastic pemphigus. Even with DIF analysis. 7 cases could not be definitively assigned a particular cause. DIF analysis is not only proving very useful for differential diagnosis, but also adds insight into possible pathogenic mechanisms of DG

Mucocutaneous features of autoimmune blistering diseases

This review will describe adult onset mucocutaneous/autoimmune diseases that involve defects in cell-to-cell, cell-to-matrix, or cell-to-basement membrane adhesion. Included in this group are pemphigus, cicatricial pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and bullous systemic lupus erythematous. Detection and treatment of blistering disorders that manifest early in the oral cavity may prevent widespread involvment of skin. During the past few years, targets of autoantibodies have been clarified and new targets have been identified, allowing better understanding of the pathophysiology involved in these diseases. New information about more effective regimens with fewer side effects has also been obtained, presenting new treatment options. Clinical manifestations and management of these disorders will be described as well as histopathologic, ultrastructural, and immunopathologic studies that distinguish each disorder and faciliatte diagnosis and treatment

Penfigoide benigno de las mucosas: reporte de caso con lesiones gingivales y expresión palmar / Benign mucous membrane pemphigoid: report of a case with gingival and palmar expressions

El penfigoide benigno de las mucosas es una enfermedad que tradicionalmente se presenta en boca. Su diagóstico y tratamiento debe ser realizado por un grupo multidisciplinario de profesionales donde intervienen periodoncistas, patólogos clínicos e histopatólogos bucales. El siguiente caso muestra un abordaje integrado de especialistas, lo que se tradujo en la resolución total de la entidad en el paciente. Se realiza revisión bibliográfica y propuesta de tratamiento

Descamative gingivitis: a clinical, histopathologic, and immunologic study

Desquamative gengivitis is believed to be a clinical sign of certain mucocutaneous diseases rather than a distinct pathologic entity. The prevelence of descamative gingivitis was studied in group of patients with most common mucocutaneous diseases. Of 414 patients with pemphigus vulgaris, mucous membrane pemphigoid, or oral lichen planus, 49 (11.8%) exhibeted gingival lesions in the form of desquamative gingivitis. DEsquamative gingivitis was most prevalent in the patients with mucous membrane pemphigoid (41.6%) followed by those with pemphigus vulgaris (9.1%). Other clinical characteristics, as well as histopathologic and immunohistochemical findings, that aid in early diagnosis are presented