No abstract available.
Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa*

BACKGROUND: In epidermolysis bullosa acquisita, it has been recognized that there exists heterogeneity in the clinical and serologic/immunopathologic features. OBJECTIVE: We examined patients with epidermolysis bullosa acquisita to see if there were any associated clinical and serological features which may predict disease activity or prognosis in the disease. METHODS: Clinical and some serologic features were compared. between 2 groups of patients with epidermolysis bullosa acquisita; one with complete remission of the symptoms and signs of the disease for more than 2 years and the other group with persistent disease activities of longer than 5 years.
Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Population Characteristics ; Prognosis

Introduction: Epidermolysis Bullosa Pruriginosa (EBP) is a rare subtype of the inherited Dystrophic ~ Epidermolysis Bullosa spectrum of diseases and results from a gene mutation in COL7AL Though predominantly an autosomal dominant disease, autosomal recessive and even sporadic have been reported. Case Summary: Case Summary:We report a case of a 12-year-old Filipino male presenting with a chronic history of numerous scratching-induced blisters predominantly distributed on the extensor aspect of his arms and legs without concomitant oral lesions, nail dystrophy, or hair findings, and without a family history of similar lesions. Histopathologic assessment, Direct Immunofluorescence (DIF), and Indirect Immunofiuorescence (IIF) showed a subepidermal split with scant inflammatory infiltrates, no immunofluorescence, and absent userrated linear immunofluorescence at the dermal-side of the Salt Split Skin slide, respectively, which were all consistent with EBP. Enzyme-Linked Immunosorbent Assay (ELISA) for Anti-Collagen VII antibodies was slightly elevated, which may suggest an alternative diagnosis of Epidermolysis Bullosa Acquisita (EBA). This slight elevation may be due to the mutated Collagen Vil protein becoming antigenic and therefore provoking an immune response. To conclusively distinguish EBP from EBA, a COL7AI gene mutation analysis was recommended. With a diagnosis of EBP cannot totally rule out EBA, the patient was initially managed with dapsone monotherapy, counseled regarding behavioral modification to reduce scratching and trauma, advised wound care and close monitoring for the development of oropharyngeal lesions, and recommended for COL7A1 genetic mutation analysis. Conclusion This report demonstrates a case of EBP with elevated Anti-Collagen VII antibodies. The diistinction between EBP and EBA is important because this changes the management: EBP is largely supportive, while EBA may benefit from immunosuppressive therapy.
Epidermolysis Bullosa Pruriginosa ; Enzyme-Linked Immunosorbent Assay ; Epidermolysis Bullosa Acquisita

We report a case of epidermolysis bullosa acquisita in a 53 year-old woman who had. extreme skin fragility, trauma induced blisters and erosions usually localized to extensor site of the skin surface, and healing with scars and milia for several years. Histologic findings of perilesional skin showed normal epidermis, subepid malblister and spirsely infiltration of inflammatory cells. Direct immuncofluorescence showed depositiori of IgCi and C3 in a linear pattern along the epidermal basement, membrane in the per ilesional skin. In indirect immuriofluorescence, using 1.0M sodium chloride separated nor mal human skin as the sntbstrate, antitodies(IgG at a titer of 1:40) were bound to dermal site of the separation, indicating that the patient serum yields the reaction in or below the lamiria densa. Electronmicroscopic findings showed the roof of the blister was located. below the basal lamiria.
Blister ; Cicatrix ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Humans ; Membranes ; Middle Aged ; Skin ; Sodium Chloride

We report a case of epidermolysis bullosa acqumta with characteristic clinical features, subepidermal vesicles in histopathology, and deposits of IgG in basement membrane zone at routine direct immunofluorescent test. 1M NaCl-treated immunofluorescent test was performed in order to correctly diagnose our case. In this method, linear immunofluorescent deposits of IgG were found only at the dermal part of separation induced by 1M NaC1 treatment to skin specimen.
Basement Membrane ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Immunoglobulin G ; Methods ; Skin

A 52-year-old man had a twenty-five year history of recurrent bullous eruption that was localized to both cheeks. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and salt-split direct immunofluorescence studies that were performed on the perilesional skin. The patient has been in partial remission state with the treatment of low dose dapsone (12.5~25 mg) and topical tacrolimus. Herein, we report on a case of EBA localized to the face, and it showed a favorable response to treatment with low-dose dapsone and topical tacrolimus.
Cheek ; Dapsone ; Epidermolysis Bullosa ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Humans ; Middle Aged ; Skin ; Tacrolimus

Introduction: Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune blistering disease which presents in the skin and mucous membranes. The decrease in anchoring fibrils in the basement membrane zone causes separation of the epidermis from the dermis, resulting in its blistering presentation. The treatment plan will depend on the severity of the disease. The first-line treatment for mild EBA includes topical corticosteroids and immunomodulators such as dapsone and colchicine; while severe cases of EBA may be given intravenous immunoglobulins, systemic steroids, and immunosuppressants such as azathioprine and cyclophosphamide. Case Report: This is a case of a 50-year-old Filipino male who presented with a 2-year history of vesicles and tense bullae which evolved into papules, plaques and erosions with scarring and milia formation on the scalp and trauma-prone areas of the trunk and extremities. Clinical examination revealed multiple, well-defined, irregularly shaped erythematous papules and plaques with crusts, scales, erosions, pearl-like milia and scarring on the chest, back, upper, and lower extremities. The oral mucosa was moist with some ulcers on the tongue. Histopathologic examination using Hematoxylin and Eosin (H&E) stain revealed the absence of the epidermis with retention of dermal papillae suggestive of subepidermal clefting. Further examination with direct immunofluorescence (DIF) revealed monoclonal immunoglobulin (IgG) deposits demonstrating an intense linear fluorescent band at the dermoepidermal junction, consistent with Epidermolysis Bullosa Acquisita. Overall, the combined administration of prednisone, azathioprine, and colchicine resulted only in transient and incomplete resolution of lesions in this case of EBA. Conclusion The management of EBA is mostly supportive with the goal of minimizing complications. Combination treatments using steroids, colchicine, and azathioprine have been reported with various results. Its management remains challenging as most cases are refractory to treatment.
Epidermolysis Bullosa Acquisita ; bullous disease ; azathioprine ; colchicine ; prednisone

The clinical spectruni of epidermolysis bullosa acquisita(EBA) is much broader than originally thought. Although the full extent of the clinical presenation is still being defined, it is now known that EBA include the followings: a non-inflammatory mechanobullous condition equating wit,h classical EBA; an inflammatory vesiculosullous eruption mimicking bullous pernphigoid; and a mucosal-centered disease with sarring similar to cicatricial pemphigoid. Among the nine cases of EBA, aged between 34 to 70 year-old, seen in recent years, three patients had mechanobullous lesions with skin fragilities and scarrings; three patients had inflammatory bullous eruptions, and three other patient had combined features of mechanobullous/inflammatory bullous lesions. Mucous membrane lesions were recognized in sex cases, and the rnos! frequent site of involvement was the oral mucosae. According to observations of these patients episodes of inflammatory bullous eruptions appeared to be present in seven cases and have been considered as early sympoms of the disease. It has been noted, however, that in two cases lesions develop d as an non-inflammatory mechanobullous from thonset. Based on the ability of EBA to mimick bullous pemphigoid or cicatricial pernphogoid and the fact that such cases have perhaps been missed, we feel EBA is more common than past literature has suggested.
Aged ; Blister ; Cicatrix ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Mouth Mucosa ; Mucous Membrane ; Pemphigoid, Benign Mucous Membrane ; Pemphigoid, Bullous ; Skin

Epidermolysis bullosa acquisita (EBA) is an uncommon autoimmune subepidermal blistering disorder and has four clinical subtypes. Among the four types of EBA, the cicatricial pemphigoid-like type is rarer than the other types and clinically the worst one. We experienced a case of cicatricial pemphigoid-like type of EBA in a 69-year-old woman, whose initial symptom was painful erosive lesions of oral mucous membrane before development of ocular and bullous cutaneous lesions. The clinical, histopathological findings and immunoblot assay were all typical of the disease. The course of her disease showed remissions by treatments including corticosteroid and intravenous immunoglobulin, but each time with exacerbations.
Aged ; Blister ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Glycogen Storage Disease Type VI ; Humans ; Immunoglobulins ; Mucous Membrane

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disorder characterized by autoantibodies to anchoring fibril (type VII) collagen. EBA is known to have a wide clinical spectrum that includes a non-inflammatory mechanobullous presentation, an inflammatory vesiculobullous eruption akin to bullous pemphigoid, and a mucosal centered disease with scarring that is reminiscent of cicatrical pemphigoid. Patients with EBA often have lesions on the oral mucosa, but esophageal involvement has not been well documented. We report a case of EBA in a 63-year-old woman who had a pseudomembranous esophageal lumen. To our knowledge, this is the first report of EBA with pseudomembranous esophageal involvement.
Autoantibodies ; Blister ; Cicatrix ; Collagen ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Esophagus ; Female ; Humans ; Middle Aged ; Mouth Mucosa ; Pemphigoid, Bullous