Epidermolysis bullosa acquisita is caused by antibodies targeting type vii collagen, the major component of anchoring fibrils that connect the basement membrane to. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Department of dermatology, university of iowa, va medical center, iowa city, iowa. Epidermolysis bullosa university of kansas medical center. The early published cases 14 were diagnosed by the clinical appearance of. Acquired forms of epidermolysis bullosa have been recognized since 1895. In deb there is a hereditary defect in the gene that encodes type vii collagen c7, the major component of anchoring fibrils. Read medical definition of eba epidermolysis bullosa acquisita. Lesions occur predominantly on areas of trauma and often heal with scarring, like cp. Epidermolysis bullosa acquisita eba is a chronic autoimmune subepidermal blistering disease of the skin and mucus membranes. Epidermolysis bullosa eb is a rare disorder that causes the skin and sometimes surfaces inside the body like the intestines to break and blister easily. Oct 05, 2011 epidermolysis bullosa acquisita eba was described for a century ago by ellliot.
The exact etiology of epidermolysis bullosa acquisita is not known, but data suggest it involves autoimmune antibodies directed against type vii collagen, a key component of the anchoring fibrils within the dermalepithelial junction. Eba can occur at any age but more frequently affects elderly persons. Epidermolysis bullosa acquisita is characterized clinically by blisters, scars, and milia primarily at the traumaprone areas. It typically affects the hands and feet, and is typically inherited in an autosomal dominant manner, affecting the keratin genes krt5 and krt14. Epidermolysis bullosa acquisita in a great dane hill. Epidermolysis bullosa acquisita eba is a rare autoimmune blistering disease in which tense subepithelial blisters appear at sites of trauma. Epidermolysis bullosa acquisita definition nci a chronic autoimmune inflammatory disorder characterized by the formation of subepidermal blisters in the skin and the mucous membranes. Epidermolysis bullosa acquisita is an acquired autoimmune bullous disease characterized by tense blisters arising on inflamed or normal appearing skin at traumaexposed sites. Learn more about the symptoms, causes, diagnosis, and treatment of epidermolysis bullosa. Unlike eb, eba is not inherited and usually presents in adult.
Summary background epidermolysis bullosa acquisita eba is a rare pemphigoid disease involving autoantibodies to type vii collagen. Successful treatment of refractory epidermolysis bullosa. Epidermolysis bullosa eb is the name given to a group of inherited blistering diseases that are present from birth what is epidermolysis bullosa acquisita. Epidermolysis bullosa acquisita eba this type of epidermolysis bullosa is a rare type of disease that usually presents as an obtained form of mechanobullous disorder. Epidermolysis bullosa acquisita genetic and rare diseases. Noninflammatory or mildly inflammatory eba affecting only traumaprone skin the classic form may cause. Broad serologic screening is recommended unless a specific immunobullous skin disease type is suspected. Click on the image or right click to open the source website in a new browser window. The underlying cause of epidermolysis bullosa acquisita eba is not known. Pdf epidermolysis bullosa acquisita associated with psoriasis.
Epidermolysis bullosa acquisita is a rare, acquired, autoimmune subepidermal blistering disease of the skin, characterised by blisters and erosions, especially in traumaprone sites and extensor. Mucosal morbidity in patients with epidermolysis bullosa. Eba blisters tend to be localised to areas that are easily injured such as the hands, feet, knees, elbows, and buttocks. Epidermolysis bullosa acquisita eba is a rare subepidermal autoimmune bullous disease aibd characterized by immune deposits on anchoring fibrils afs of cutaneous and mucosal basement membrane zones bmz. The disease is rare, with an estimated annual incidence of 0. It is thought to be an autoimmune disorder, which means that the immune system attacks healthy cells by mistake. Dec 21, 2018 home medterms medical dictionary az list eba epidermolysis bullosa acquisita definition medical definition of eba epidermolysis bullosa acquisita medical author. Mayuzumi m, akiyama m, nishie w, ukae s, abe m, sawamura d, hashimoto t, shimizu h. Epidermolysis bullosa acquisita eba is an autoimmune blistering disease that is mediated by antibodies against collagen vii, which is the major component of the anchoring fibrils in the lamina densa of the basement membrane.
Manz3, enno schmidt1,2, detlef zillikens1,2 and ralf j. Pdf epidermolysis bullosa acquisita associated with. In eba the body attacks its own anchoring fibrils collagen fibrils that insert. Epidermolysis bullosa acquisita panel university of utah. Epidermolysis bullosa acquisita eba is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type vii collagen c7 structures, a major. Ludwig1,2 epidermolysis bullosa acquisita eba is a prototypic. Familial epidermolysis bullosa acquisita megan h noe 1, mei chen phd 2, david t woodley md 2, janet a fairley md 3 dermatology online journal 14 12. Epidermolysis bullosa acquisita is a chronic subepidermal blistering disease associated with autoimmunity to type vii collagen within anchoring fibril structures that are located at the dermoepidermal junction. Epidermolysis bullosa acquisita eba is an autoim mune mechanobullous disease that is clinically charac terised by blisters and erosions on. Ludwig1,2 epidermolysis bullosa acquisita eba is a prototypic organspeci. This report describes the first case of one such disease, epidermolysis bullosa acquisita, to be documented in the uk. Emerging treatment for epidermolysis bullosa acquisita. The stiff blisters develop at sites of trauma which are vulnerable to injury such as the hands, elbows, knees, feet, and buttocks.
Epidermolysis bullosa acquisita is a rare, acquired, chronic condition characterized by subepidermal blistering. Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type vii collagen. Eba is a rare blistering disease which produces deep, tense blisters on the skin and mucosal surfaces mouth, genitals, nose and eyes which typically heal with scarring. Epidermolysis bullosa acquisita description, causes and risk factors. Eba is characterized by the presence of autoantibodies against type vii collagen which is a major component of the anchoring fibrils at the dermalepidermal junction. Eba is classically described as a mechanobullous disorder characterized by skin fragility, noninflammatory tense bullae, milia, and scarring. Four scoring systems have been proposed as potential outcome measures for eb, including the japanese indices, 1 the birmingham eb severity bebs score, 2 the instrument for scoring clinical outcome of research for epidermolysis bullosa iscoreb 3 and the epidermolysis bullosa disease activity and scarring index ebdasi. Epidermolysis bullosa acquisita in a great dane hill 2007. Epidermolysis bullosa acquisita eba is a rare, chronic autoimmune blistering disease. Patients with eba suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Epidermolysis bullosa acquisita is caused by antibodies targeting type vii collagen, the major component of anchoring fibrils that connect the basement membrane to dermal structures. A characteristic feature of all types of eb is the presence of recurrent blistering or erosions, the result of even minor traction to these tissues. Epidermolysis bullosa acquisita dermatologic disorders. Department of dermatology, university of iowa, iowa city, iowa 2.
Epidermolysis bullosa acquisita kim 20 journal of the. Epidermolysis bullosa acquisita eba is a rare autoimmune disorder that causes the skin to blister in response to minor injury. Patients with epidermolysis bullosa acquisita eba should have a negative family and personal history of blistering disorders in order to make the diagnosis. Epidermolysis bullosa acquisita eba was described for a century ago by ellliot. Inflammatory epidermolysis bullosa acquisita effectively treated. Epidermolysis bullosa acquisita eba is a rare, immunobullous disease, characterized by circulating and tissuebound antibodies against type vii collagen c7 of anchoring fibrils in the. It is one of the rarest subepidermal bullous conditions in western europe with an incidence of about 0. Epidermolysis bullosa acquisita eba is an orphan autoimmune disease. Feb 27, 2014 epidermolysis bullosa acquisita eba is a rare autoimmune disorder that causes the skin to blister in response to minor injury. Epidermolysis bullosa acquisita panel ordering recommendation this is an autoantibody panel for initial diagnostic assessment and disease monitoring in in disorders that present with blistering and erosions, which affect skin and mucous membranes, from suspected epidermolysis bullosa acquisita. Treatment is with corticosteroids, dapsone, and meticulous skin care. Inflammatory epidermolysis bullosa acquisita eba is a rare clinical variation of eba with acute onset of erythemaurticaria and vesiclebullous lesions with.
Epidermolysis bullosa acquisita dermatology education. Unlike eb, eba is not inherited and usually presents in adult life. Epidermolysis bullosa acquisita what is epidermolysis bullosa acquisita eba. The target for epidermolysis bullosa acquisita autoantibodies is type vii collagen localized to anchoring fibrils just. Inflammatory epidermolysis bullosa acquisita eba is a rare clinical variation of eba with acute onset of erythemaurticaria and vesiclebullous lesions with marked pruritus 1. Four patients with documented epidermolysis bullosa acquisita were evaluated by a multidisciplinary team of care providers 4 dermatologists, an ophthalmologist, a radiologist, a voice and speech specialist, and an otolaryngologist for 1 to 5 years to characterize mucosal involvement and its complications and response to treatment. Epidermolysis bullosa acquisita an overview sciencedirect. Previous biopsies performed at an outside facility showed findings consistent with bullous drug eruption and immunobullous disorders. This is a pdf file of an unedited manuscript that has been. Of all the clinical types of epidermolysis bullosa, only epider molysis bullosa acquisita eba. Acantholysis bullosa, acanthosis bullosa, bullosa hereditaria, dominant and recessive epidermolysis bullosa, dowlingmeara syndrome, epidermolysis bullosa acquisita, epidermolysis bullosa hereditaria, epidermolysis bullosa letalias, epidermolysis bullosa letalis, epidermolysis bullosa simplex, epidermolysis hereditaria tarda, goldscheiders disease, hallopeausiemens disease, heinrichsbauer. Read medical definition of epidermolysis bullosa acquisita. Epidermolysis bullosa acquista eba is an acquired, subepidermal bullous disease with clinical features similar to the genetic forms of dystrophic epidermolysis bullosa deb. Epidermolysis bullosa acquisita medical definition.
Mar 03, 2014 symptoms of epidermolysis bullosa acquisita eba usually occur in a persons 30s or 40s. Treatment of epidermolysis bullosa acquisita eba is difficult, and most treatment regimens are based on anecdotal reports. This is a mechanobullous subepidermal autoimmune blistering disorder that usually begins in adulthood. Systemic corticosteroids have been the mainstay of therapy for severe or extensive disease but impose an increased risk for postoperative complications including surgical site infections. In eba, certain immune proteins usually igg autoantibodies mistakenly target and attack a specific type of collagen a skin protein involved in anchoring the skin. The epidermolysis bullosa disease activity and scarring. Pediatric inflammatory epidermolysis bullosa acquisita in. Epidermolysis bullosa simplex is a form of epidermolysis bullosa that causes blisters at the site of rubbing. Indirect immunofluorescence is positive in about 50% of cases. Bullous pemphigoid mimicking epidermolysis bullosa acquisita. Carver college of medicine department of dermatology 200 hawkins drive 40025 pomerantz family. Epidermolysis bullosa acquisita primary care dermatology. Inherited epidermolysis bullosa eb encompasses over 30 phenotypically or genotypically distinct entities which share as a common feature mechanical fragility of epithelial lined or surfaced tissues, most notably the skin.
Epidermolysis bullosa news is strictly a news and information website about the disease. Epidermolysis bullosa eb is a group of inherited diseases that are characterised by blistering lesions on the skin and mucous membranes. It has the same classical progression, including the possibility of milia formation upon healing 1. Epidermolysis bullosa acquisita is an acquired chronic blistering disease of the skin, in which separation of the skin occurs in the basementmembrane zone between the epidermis and the dermis. Therefore, there is a failure in keratinisation, which affects the integrity and the ability of the skin to resist mechanical stresses. Epidermolysis bullosa acquisita eba is an autoimmune subepidermal bullous disorder of the skin and mucous membranes.
Therefore, eba is a prototypical autoimmune disease with a wellcharacterized pathogenic relevance of autoantibody binding to the target antigen. Dec 21, 2018 home medterms medical dictionary az list epidermolysis bullosa acquisita definition medical definition of epidermolysis bullosa acquisita medical author. The pathogenic relevance of autoantibodies targeting type vii collagen col7 has been welldocumented. Clinical presentation, pathogenesis, diagnosis, and. Razzaque ahmed, mdb boston, massachusetts epidermolysis bullosa acquisita eba is a rare, chronic, subepidermal, mucocutaneous blistering disease characterized by skin fragility and spontaneous as well as traumainduced blisters that heal with scar formation and milia. Epidermolysis bullosa acquisita clinical presentation. It is due to circulating autoantibodies ab directed to type vii collagen c7. Since then, refinements in reagents and immunological techniques have allowed diseases which are histologically similar but which have a different molecular pathogenesis to be described. Epidermolysis bullosa pictures, treatment, life expectancy. Clinical presentation, pathogenesis, diagnosis, and treatment of.
Bullous pemphigoid mimicking epidermolysis bullosa acquisita liza gill, md case. Epidermolysis bullosa eb is the name given to a group of inherited blistering diseases that are present from birth. These may occur anywhere on the body but most commonly appear at sites of friction and minor trauma such as the feet and hands what is epidermolysis bullosa simplex. Epidermolysis bullosa acquisita eba is an acquired subepithelial blistering disease of the skin and mucous membranes mediated by igg autoantibodies against type vii collagen otoole and woodley, 2000. Epidermolysis bullosa acquista eba is an acquired, subepidermal bullous disease with. Epidermolysis bullosa acquisita medigoo medical tests. Carver college of medicine department of dermatology 200 hawkins drive 40025 pomerantz family pavilion iowa city, ia 522421089. Epidermolysis bullosa is a rare and painful skin disorder has no cure. Epidermolysis bullosa acquisita is a chronic blistering disease of skin and mucous membranes characterized by subepidermal blisters and tissuebound as well as circulating autoantibodies to the dermal epidermal junction. Epidermolysis bullosa acquisita can occur in all ages. Mar 05, 2019 epidermolysis bullosa acquisita eba is a chronic autoimmune subepidermal blistering disease of the skin and mucus membranes. It does not provide medical advice, diagnosis or treatment. From pathophysiology to novel therapeutic options michael kasperkiewicz1, christian d.
Epidermolysis bullosa acquisita medical definition merriam. Epidermolysis bullosa acquisita is a subepidermal blistering disease associated with predominantly igg autoantibodies that bind the dermal side of saltsplit skin. Epidermolysis bullosa treatment epidermolysis bullosa news. In eba the body attacks its own anchoring fibrils collagen fibrils that insert in to the basal lamina of the epidermis and bind it. Epidermolysis bullosa an overview sciencedirect topics. Epidermolysis bullosa acquisita eba is a rare, sporadic, subepithelial, mucocutaneous blistering disease that usually develops in adulthood. Considering the initial inflammatory pattern and onset in adult age, it is necessary to make differential. Diagnosis is by skin biopsy and direct immunofluorescence. These images are a random sampling from a bing search on the term epidermolysis bullosa acquisita. A rare, chronic, incurable, sub epithelial autoimmune bullous disease characterized by the presence of.
Epidermolysis bullosa acquisita a pemphigoid like disease. Current treatment options rely on nonspecific immunosuppression, which in many cases, does not lead to a remission of treatment. Epidermolysis bullosa acquisita eba is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. Epidermal bullosa acquisita department of dermatology. Alternatively, eba can present as an inflammatory bullous. Jan 30, 2015 epidermolysis bullosa acquisita eba is a rare subepidermal autoimmune bullous disease aibd characterized by immune deposits on anchoring fibrils afs of cutaneous and mucosal basement membrane zones bmz. The signs and symptoms can differ among affected people, and the condition has several distinct forms of onset. Inherited epidermolysis bullosa orphanet journal of rare. Images in clinical medicine from the new england journal of medicine epidermolysis bullosa acquisita. Clinical presentation, pathogenesis, diagnosis, and treatment. Epidermolysis bullosa acquisita eba is a chronic mucocutaneous autoimmune skin blistering disease. Medical definition of epidermolysis bullosa acquisita. Common areas of blistering include the hands, feet, knees, elbows, and buttocks. This leads to pain, discomfort, and sometimes fatal complications.
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