Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Penfigoide cicatricial, causa poco común de estenosis supraglótica / Cicatricial pemphigoid: uncommon cause of supraglottic stenosis

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.

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.

Response to levamisole and low-dose prednisolone in 41 patients with chronic oral ulcers: a 3-year open clinical trial and follow-up study

Objective. The purpose of this open clinical trial and follow-up study was to evaluate the short-term and long-term clinical efficacy of levamisole used with low-dose prednisolone in 30 patientes with oral lichen planus, 6 patients with erythema multiforme, 3 patients with mucous membrane pemphigoid, and 2 patients with early pemphigus vulgaris. Study design. All patients were given 150 mg/day of levamisole and 15 mg/day of prednisolone for 3 consecutive days each week, along with topically applied dexamethasone orobase (dexaltin). Results. Twenty-three patients showed dramatic remission of signs symptoms within 2 weeks; 18 patients experimenced partial remission. Forty patients reported significant pain relief, and almost none showed evidence of oral ulcerative lesions after 4 to 8 weeks of treatment. In contrat, 1 patient with oral lichen planus with allergy to levamisole reported a partial response from prednisolone alone. All 29 patients with oral lichen planus remained free from symptoms for more than 6 months. All 6 patients with erythema multiforme, all 3 patients with mucous membrane pemphigoid, and bot patients with pemphigus vulgaris also remained free from symptoms for 3 to 3 years. There were few side effects from the teatment; there applied dexaltin in the treatment of diffuse atrophic or ulcerative gingivitis. Conclusions. The addition of levamisole to prednisolone may produce improved results in the management of orosive lichen planus, erythema multiforme, mucous membrane pemphigoid, and early pemphigus vulgaris

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

Alteraçöes dos tecidos moles da boca. Parte 1: doenças vesicobolhosas, condiçöes ulcerativas e lesöes verrucosas papilares / Alterations of bucal soft tissues. Part 1: vesicobullous diseases, ulcerative conditions and verrucous papilars lesions

Os autores iniciam o desenvolvimento do tema alteraçoes de tecidos moles da boca, esclarecendo à classe médica sobre as principais doenças vesicobolhosas, condiçoes ulcerativas e lesoes verrucosas papilares.