ABSTRACT
Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may be another cause of prerenal kidney failure. Further studies are needed to verify our hypothesis.
Subject(s)
Acute Kidney Injury/etiology , Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/pathology , Acute Kidney Injury/drug therapy , Aged , Biopsy , Epidermolysis Bullosa Acquisita/drug therapy , Fluorescent Antibody Technique, Direct , Humans , Male , Skin/pathology , Treatment OutcomeABSTRACT
Abstract: Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may be another cause of prerenal kidney failure. Further studies are needed to verify our hypothesis.
Subject(s)
Humans , Male , Aged , Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/pathology , Acute Kidney Injury/etiology , Skin/pathology , Biopsy , Epidermolysis Bullosa Acquisita/drug therapy , Treatment Outcome , Fluorescent Antibody Technique, Direct , Acute Kidney Injury/drug therapyABSTRACT
Epidermolysis bullosa acquisita (EBA) is a rare auto-immune dermatologic disease, produced by auto-antibodies against colagen VII. We report a 44 years old male patient with EBA diagnosed 15 years before, who presented with progressive disphagia, being diagnosed an esophageal involvement of EBA. The patient was submitted to endoscopic treatment with thermoplastic bougie dilation and intralesional corticosteroid injection. The patient improved clinically with recovery of nutritional status. Esophageal involvement in EBA is very rare and its reason is still unknown. Endoscopic approach must be cautiously performed with the use of small diameter endoscopes, small caliber dilators, intralesional injection of corticosteroid and enteral tube in order to minimize the risks of complications, as well as esophageal rest from food trauma and better reparatory molding of the epithelium.
Subject(s)
Epidermolysis Bullosa Acquisita/complications , Esophageal Stenosis/etiology , Esophageal Stenosis/therapy , Esophagoscopy , Glucocorticoids/administration & dosage , Prednisone/administration & dosage , Adult , Combined Modality Therapy , Dilatation/instrumentation , Humans , Injections, Intralesional , MaleABSTRACT
La epidermolisis ampollar adquirida (EAA) es una enfermedad autoinmune provocada por autoanticuerpos contra el colágeno tipo VII de la sublámina densa de la dermis. Sus manifestaciones clínicas exigen especial atención para diferenciarla del lupus ampollar (LA), el penfigoide ampollar (PA) y la porfiria cutánea tarda (PCT). La clínica, la histopatología, la inmunofluorescencia directa (IFD) y la inmunofluorescencia indirecta (IFI) con la técnica de salt-split son de gran ayuda para el diagnóstico. El caso de una paciente con EAA y lupus eritematoso sistémico (LES) plantea la discusión sobre las diferencias y semejanzas entre el LA y la EAA. La presencia de mecanismos inmunopatogénicos comunes y la asociación de LES y EAA permitiría suponer que se trata de expresiones clínicas de una misma afección
Subject(s)
Humans , Adult , Female , Epidermolysis Bullosa Acquisita/diagnosis , Autoantibodies , Collagen , Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/drug therapy , Lupus Erythematosus, SystemicABSTRACT
La epidermolisis ampollar adquirida (EAA) es una enfermedad autoinmune provocada por autoanticuerpos contra el colágeno tipo VII de la sublámina densa de la dermis. Sus manifestaciones clínicas exigen especial atención para diferenciarla del lupus ampollar (LA), el penfigoide ampollar (PA) y la porfiria cutánea tarda (PCT). La clínica, la histopatología, la inmunofluorescencia directa (IFD) y la inmunofluorescencia indirecta (IFI) con la técnica de salt-split son de gran ayuda para el diagnóstico. El caso de una paciente con EAA y lupus eritematoso sistémico (LES) plantea la discusión sobre las diferencias y semejanzas entre el LA y la EAA. La presencia de mecanismos inmunopatogénicos comunes y la asociación de LES y EAA permitiría suponer que se trata de expresiones clínicas de una misma afección (AU)
Subject(s)
Humans , Adult , Female , Epidermolysis Bullosa Acquisita/diagnosis , Epidermolysis Bullosa Acquisita/drug therapy , Epidermolysis Bullosa Acquisita/complications , Collagen , Autoantibodies , Lupus Erythematosus, SystemicABSTRACT
PURPOSE: To report clinical and laboratory findings of bilateral corneal involvement in a patient with epidermolysis bullosa acquisita. METHODS: A 25-year-old man with a history of progressive and painless loss of vision in both eyes presented to our service with bilateral corneal involvement: peripheral corneal perforation in one eye and advanced corneal thinning in the other eye. There was concomitant dermatologic bullous disease. Clinical and laboratory exams were analyzed. RESULTS: The patient was diagnosed as having epidermolysis bullosa acquisita. Therapeutic corneal patch graft and conjunctival resection with cryotherapy were done, with satisfactory results. CONCLUSION: Bilateral corneal involvement in epidermolysis bullosa acquisita is described. To the best of our knowledge, this is the first description of such a case. Surgical management of the ocular findings associated with systemic therapy with colchicine seems to be a good therapeutic option in the management of this defying disease.