ABSTRACT
Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.
Subject(s)
Humans , Male , Adult , Immunoglobulin E/blood , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/pathology , Desmogleins/immunology , Keratosis/immunology , Keratosis/pathology , Skin/pathology , Autoantibodies/blood , Autoantigens/blood , Biopsy , Methylprednisolone/therapeutic use , Pemphigoid, Bullous/drug therapy , Non-Fibrillar Collagens/blood , Pressure Ulcer/pathology , Glucocorticoids/therapeutic use , Keratosis/drug therapyABSTRACT
Literature data analysis, providing an exact explanation of the lichen planus pathogenesis, as well as its transition into other rare forms such as Keratosis lichenoides chronica or Graham Lassueur Piccardi Little Syndrome are scant, or totally missing. The chronological course of the disease, known in the literature as lichen planus, varies. Some patients develop Lichen planus or lichen nitidus and there is no logical explanation why. It is also not clear why single patients initially develop ulcerative lesions in the area of the mucosa and only in a few of them these lesions affect the skin. Antigen Mimicry and Epitope Spreading could be the possible pathogenic inductor in cases of lichenoid dermatoses, as well as the cause for their transition into ulcerative, exanthematous or other rare forms. The Epitope Spreading is probably not the leading pathogenetic factor in lichen planus but a phenomenon which occurs later. This manuscript analyzes some basic pathogenic aspects and presents some possible medical hypotheses regarding the heterogenic clinical picture and pathogenesis of lichen planus and lichenoid like pathologies of the skin which, in the near future should be analyzed in details in order to clarify several dilemmas the clinical dermatologist has to face.
Análises das informações disponíveis na literatura que forneçam uma explicação precisa sobre a patogênese do Líquen Plano, assim como sobre sua transição para outras formas raras da doença, como Ceratose Liquenóide Crônica ou Síndrome de Graham-Little-Piccardi- Lassueur , são raras ou inexistentes. O curso cronológico da doença, conhecida na literatura como Líquen Plano, varia. Alguns pacientes desenvolvem Líquen Plano ou Líquen Nítido e não ha uma explicação lógica do por quê. Também não está claro por que alguns pacientes inicialmente desenvolvem lesões ulcerativas na área da mucosa e em apenas alguns deles essas lesões afetam a pele. Mimetismo Antigênico ou Espalhamento de Epítopos poderiam ser fatores patogênicos indutores em casos de Dermatoses Liquenóides, e também fatores responsáveis pela transição para a forma ulcerativa, exantematosa ou outras formas raras da doença. Espalhamento de Epítopos provavelmente não é o principal fator patogênico envolvido no Líquen Plano, mas um fenômeno de ocorrência posterior.Esse manuscrito analisa alguns aspectos patogênicos básicos e apresenta algumas hipóteses médicas sobre o quadro clínico heterogênico e a patogênese do Líquen Plano e de patologias da pele do tipo liquenóide. Essas patologias devem, em um futuro próximo, ser analisadas minuciosamente a fim de esclarecer vários dilemas que o dermatologista clínico tem de enfrentar.