Subject(s)
Pemphigoid, Benign Mucous Membrane , Pemphigus , Humans , Mouth Diseases/epidemiology , Mouth Mucosa/pathology , Pemphigoid, Benign Mucous Membrane/epidemiology , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigus/epidemiology , Pemphigus/diagnosis , PrevalenceABSTRACT
Ectodermal dysplasia (ED) is a group of several genetic conditions with absence or dysgenesis of at least two ectodermal derivatives: teeth, skin and its appendages including hair, nails, eccrine and sebaceous glands. The most important clinical findings in patients with ED are hypodontia, hypotrichosis, and hypohidrosis, which can lead to episodes of hyperthermia. Few reports have focused on the progressive keratopathy in ED. Cicatrizing conjunctivitis associated with anti-basement membrane autoantibodies has been described. We report a series of three ectodermal dysplasia patients with an ocular phenotype typically seen in ocular mucous membrane pemphigoid; conjunctival immunohistopathology revealed anti-basement membrane autoantibodies in all of them, and systemic immunosuppression proved to be effective in improving symptoms and helping to stabilize ocular surface disease.
Subject(s)
Autoantibodies/immunology , Basement Membrane/immunology , Conjunctiva/pathology , Ectodermal Dysplasia/immunology , Pemphigoid, Benign Mucous Membrane/diagnosis , Basement Membrane/pathology , Conjunctiva/immunology , Ectodermal Dysplasia/diagnosis , Ectodermal Dysplasia/etiology , Female , Humans , Middle Aged , Pemphigoid, Benign Mucous Membrane/complications , Pemphigoid, Benign Mucous Membrane/immunologyABSTRACT
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
Subject(s)
Humans , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/therapy , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigoid, Benign Mucous Membrane/pathology , Pemphigoid, Benign Mucous Membrane/therapy , Pemphigus/diagnosis , Pemphigus/immunology , Pemphigus/therapy , Skin Diseases, Vesiculobullous/classification , Skin Diseases, Vesiculobullous/diagnosis , Skin Diseases, Vesiculobullous/immunology , Skin Diseases, Vesiculobullous/pathology , Autoimmune Diseases/classification , Autoimmune Diseases/complications , Autoimmune Diseases/pathology , Fluorescent Antibody TechniqueABSTRACT
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
Subject(s)
Humans , Female , Adolescent , Gingival Diseases/etiology , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Biopsy , Dermatitis, Contact/diagnosis , Dermatitis, Contact/etiology , Dexamethasone/therapeutic use , Diagnosis, Differential , Fluorescent Antibody Technique, Direct , Patient Care Team , Pemphigoid, Benign Mucous Membrane/epidemiology , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigoid, Benign Mucous Membrane/pathologyABSTRACT
Cell/cell and cell/matrix adhesion proteins are responsible for maitaining the integrity of the mucosal lining of the oral cavity. Disease processes that destroy keratinocytes or adversely affect their adhesion to one another or to the subjacent basement membrane will result in erosions, ulcerations, and desquamations. Immunologic processes that have a deleterious effect on the integrity of the epithelial/basement membrane/submucosa complex are reviewed, and current research findings with respect to pathogenesis are discussed. In particular, T-cell-mediated hypersensitivity is involved in recurrent aphthous stomatitis and lichen planus; humoral-mediated immunity to cadherin intercellular adhesion molecules in important in the process of acantholysis in pemphigus vulgaris, and genetic defects and antibody-mediated processes give rise to junctional separation epidemolysis bullosa and mucous membrane pemphigoid, respectively. An immune complex mechanism appears to underline the pathogenesis of eryhtema multiforme
Subject(s)
Humans , Acantholysis/immunology , Epidermolysis Bullosa/immunology , Epidermolysis Bullosa/pathology , Erythema Multiforme/immunology , Erythema Multiforme/pathology , Stomatitis, Aphthous/immunology , Stomatitis, Aphthous/pathology , Lichen Planus/immunology , Lichen Planus/pathology , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigoid, Benign Mucous Membrane/pathology , Pemphigus/immunology , Antibody Formation/physiology , Antibody Formation/immunology , Immunity, Cellular/physiology , Immunity, Cellular/immunologyABSTRACT
E estudado um caso de epidermolise bolhosa adquirida (Penfigoide bolhoso) em um homem adulto com insucesso terapeutico. E feita uma revisao da entidade e estudadas as relacoes com o penfigoide bolhoso o e penfigoide cicatricial. Discute-se a autonomia da entidade e destaca-se, sobretudo, como de valor diagnostico o nivel da clivagem-sublamina densa
Subject(s)
Epidermolysis Bullosa/pathology , Epidermolysis Bullosa/diagnosis , Epidermolysis Bullosa/immunology , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/immunology , Diagnosis, Differential , Prednisone/therapeutic useABSTRACT
E estudado um caso de epidermolise bolhosa adquirida (Penfigoide bolhoso) em um homem adulto com insucesso terapeutico. E feita uma revisao da entidade e estudadas as relacoes com o penfigoide bolhoso o e penfigoide cicatricial. Discute-se a autonomia da entidade e destaca-se, sobretudo, como de valor diagnostico o nivel da clivagem-sublamina densa