ABSTRACT
Epidermolysis bullosa acquisita (EBA) is an acquired chronic autoimmune blistering skin disease that rarely affects mucous membranes. We report a 75-year-old female patient who had EBA restricted to oral, nasal and laryngeal mucous membranes with life-threatening laryngeal stenosis.
Subject(s)
Epidermolysis Bullosa Acquisita/diagnosis , Laryngostenosis/diagnosis , Tracheal Stenosis/diagnosis , Aged , Colchicine/therapeutic use , Combined Modality Therapy , Drug Therapy, Combination , Epidermolysis Bullosa Acquisita/pathology , Epidermolysis Bullosa Acquisita/surgery , Female , Humans , Immunoglobulin G/metabolism , Laryngostenosis/pathology , Laryngostenosis/surgery , Laser Therapy , Microscopy, Fluorescence , Prednisolone/therapeutic use , Skin/pathology , Tracheal Stenosis/pathology , Tracheal Stenosis/surgerySubject(s)
Epidermolysis Bullosa Acquisita/epidemiology , Hemophilia A/epidemiology , Tracheostomy , Autoimmune Diseases , Comorbidity , Epidermolysis Bullosa Acquisita/immunology , Epidermolysis Bullosa Acquisita/surgery , Female , Hemophilia A/immunology , Humans , Laryngeal Diseases/immunology , Middle Aged , Mouth Diseases/immunologyABSTRACT
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.
Subject(s)
Corneal Diseases/etiology , Epidermolysis Bullosa Acquisita/complications , Adult , Corneal Diseases/pathology , Corneal Diseases/surgery , Epidermolysis Bullosa Acquisita/pathology , Epidermolysis Bullosa Acquisita/surgery , Humans , Male , Vision Disorders/etiology , Vision Disorders/pathology , Vision Disorders/surgery , Visual AcuityABSTRACT
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Subject(s)
Humans , Photopheresis/methods , Photopheresis/adverse effects , Myxedema/surgery , Scleredema Adultorum/surgery , Arthritis, Rheumatoid/surgery , Graft Rejection/surgery , Psoriasis/surgery , Arthritis, Psoriatic/surgery , Pemphigus/surgery , Multiple Sclerosis/surgery , Lichen Planus/surgery , Mixed Connective Tissue Disease/surgery , Epidermolysis Bullosa Acquisita/surgery , AIDS-Related Complex/surgeryABSTRACT
BACKGROUND: Epidermolysis bullosa acquisita (EBA) is an uncommon, acquired, chronic subepidermal bullous disease. This report describes a case of EBA with gingival involvement. A 43-year-old woman with EBA was referred to our clinic for periodontal therapy because of gingival tenderness and bleeding. She has been on cyclosporin A therapy for the last 2 years. METHODS: Clinical findings were analyzed. Anterior gingivectomy operations were performed in 2 stages. The samples obtained during the surgery were examined using histopathologic, immunohistologic, and electronmicroscopic methods. Long-term effects of the surgical periodontal treatment on gingiva were evaluated both clinically and microscopically. RESULTS: The dentition displayed minimal enamel hypoplasia. Decayed, missing, and filled surfaces score was found to be elevated. Periodontal examination showed generalized diffuse gingival inflammation and gingival enlargement localized mainly to the anterior region. Nikolsky's sign was positive. However, wound healing was uneventful after the operations. Microscopic findings were similar to those obtained from the skin. Twenty-one months after the operations, Nikolsky's sign was negative and no remarkable gingival inflammation was noted. Microscopic examination revealed that the blisters were fewer in number and smaller in size. CONCLUSIONS: These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries.
