Epidermolysis Bullosa Acquisita (Acquired Epidermolysis Bullosa)

Form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. Direct immunofluorescence shows IMMUNOGLOBULIN G deposited at the dermo-epidermal junction.

Also Known As:

Networked: 372 relevant articles (11 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Vesiculobullous Skin Diseases (Subcorneal Pustular Dermatosis)
2.	Lichen Planus (Lichen Ruber Planus)
3.	Atopic Dermatitis (Atopic Eczema)
4.	Pyoderma Gangrenosum
5.	Psoriasis (Pustulosis Palmaris et Plantaris)

Experts

1.	Zillikens, Detlef: 39 articles (09/2015 - 06/2002)
2.	Schmidt, Enno: 23 articles (09/2015 - 06/2002)
3.	Ludwig, Ralf J: 22 articles (09/2015 - 01/2011)
4.	Hashimoto, Takashi: 15 articles (11/2015 - 07/2002)
5.	Sitaru, Cassian: 15 articles (09/2014 - 06/2002)
6.	Chen, Mei: 13 articles (02/2012 - 05/2002)
7.	Zillikens, D: 11 articles (02/2013 - 01/2000)
8.	Woodley, David T: 11 articles (02/2012 - 05/2002)
9.	Recke, Andreas: 8 articles (09/2015 - 01/2011)
10.	Jonkman, Marcel F: 8 articles (06/2015 - 12/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Epidermolysis Bullosa Acquisita:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 11/01/1994 - "Epidermolysis bullosa acquisita: efficacy of high-dose intravenous immunoglobulins." 08/01/1993 - "Epidermolysis bullosa acquisita: efficacy of high-dose intravenous immunoglobulins." 03/01/2015 - "Effects of intravenous immunoglobulins on mice with experimental epidermolysis bullosa acquisita." 01/01/2002 - "Epidermolysis bullosa acquisita: treatment with intravenous immunoglobulins." 05/01/1995 - "Successful treatment of epidermolysis bullosa acquisita using intravenous immunoglobulins."
2.	Collagen Type VIIIBA 06/01/2002 - "This study sought to determine if type VII collagen, the epidermolysis bullosa acquisita autoantigen, was present in normal human colon by western blotting and immunofluorescence. " 05/01/2014 - "Epidermolysis bullosa acquisita (EBA) is a rare acquired blistering disorder caused by production of auto-antibody directed against type-VII collagen. " 07/01/2013 - "The type VII collagen (coll VII) enzyme-linked immunosorbent assay (ELISA) has been reported to have high sensitivity (> 93%) and specificity (> 96%) for diagnosing epidermolysis bullosa acquisita (EBA) in patients who are seropositive on indirect immunofluorescence on salt-split skin (SSS). " 07/01/2013 - "Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis." 11/15/2011 - "Immunization of mice with type VII collagen, an adhesion protein expressed at the cutaneous basement membrane, induces experimental epidermolysis bullosa acquisita (EBA). "
3.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/1990 - "Intractable epidermolysis bullosa acquisita: efficacy of cyclosporin A." 03/01/2001 - "Epidermolysis bullosa acquisita responsive to cyclosporin therapy." 06/01/1991 - "Clearing of epidermolysis bullosa acquisita with cyclosporine." 03/01/1990 - "Clearing of epidermolysis bullosa acquisita with cyclosporine." 11/01/1988 - "Although its mechanism of action in epidermolysis bullosa acquisita is unknown, we propose that cyclosporine may be a helpful drug for patients whose disease is refractory to more traditional forms of therapy."
4.	AutoantibodiesIBA 04/01/2014 - "Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease characterized by tissue-bound and circulating autoantibodies to the dermal-epidermal junction. " 06/01/2006 - "Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients." 06/15/2001 - "Furthermore, autoantibodies from epidermolysis bullosa acquisita patients also reacted with NC2/COL. " 05/01/1995 - "Passive transfer of autoantibodies from a patient with mutilating epidermolysis bullosa acquisita induces specific alterations in the skin of neonatal mice." 04/01/1993 - "We also found that separation of the DEJ with brief exposure of skin to 56 degrees C provided a useful substrate for testing the autoantibodies in the sera of patients with epidermolysis bullosa acquisita (EBA). "
5.	AntibodiesIBA 05/01/1990 - "In this study a variety of immunoelectron microscopic methods were used to define the precise ultrastructural binding site of epidermolysis bullosa acquisita antibodies (EBA-Ab). " 07/01/2011 - "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. " 04/01/2011 - "False-positive intercellular indirect immunofluorescence test due to anti-blood group antibodies in a patient with epidermolysis bullosa acquisita." 01/01/2011 - "Generation of antibodies of distinct subclasses and specificity is linked to H2s in an active mouse model of epidermolysis bullosa acquisita." 01/01/1998 - "Epidermal-binding circulating antibodies in epidermolysis bullosa acquisita: what are they?"
6.	ColchicineFDA LinkGeneric 02/01/2005 - "Remission of recalcitrant epidermolysis bullosa acquisita (EBA) with colchicine monotherapy." 05/01/1996 - "Colchicine for epidermolysis bullosa acquisita." 01/01/1994 - "Epidermolysis bullosa acquisita--successful treatment with colchicine." 01/01/1989 - "[Value of colchicine in treating acquired epidermolysis bullosa]." 02/01/2005 - "A 42-year-old woman with widespread recalcitrant epidermolysis bullosa acquisita (EBA) responded well to colchicine monotherapy after attempts to control her disease with other modalities failed. "
7.	Vitamin EFDA LinkGeneric 06/01/1982 - "Therapy of epidermolysis bullosa acquisita is generally regarded as difficult; following a 3-year course of high dose vitamin E therapy our patient underwent complete clearing; the possibility of a spontaneous remission, on the other hand, cannot be unequivocally ruled out." 12/01/1972 - "Therapeutic value of large doses of vitamin E in epidermolysis bullosa acquisita." 06/01/1982 - "[Epidermolysis bullosa acquisita: diagnosis by optic immunofluorescent demonstration of junctional antigens and vitamin E treatment]."
8.	Monoclonal AntibodiesIBA 02/01/2007 - "Clinical response of severe mechanobullous epidermolysis bullosa acquisita to combined treatment with immunoadsorption and rituximab (anti-CD20 monoclonal antibodies)." 09/01/1991 - "Immunofluorescence mapping was performed using polyclonal antibodies from the serum of patients with bullous pemphigold and epidermolysis bullosa acquisita and monoclonal antibodies against GB3 antigen and collagen type VII. All but the bullous pemphigold serum gave positive results; only faint and focal staining of the dermoepidermal junction was observed with bullous pemphigold serum. "
9.	Minocycline (Cyclops)FDA LinkGeneric 09/01/2014 - "Inflammatory epidermolysis bullosa acquisita effectively treated with minocycline."
10.	Gb3 antigenIBA 09/01/1991 - "Immunofluorescence mapping was performed using polyclonal antibodies from the serum of patients with bullous pemphigold and epidermolysis bullosa acquisita and monoclonal antibodies against GB3 antigen and collagen type VII. All but the bullous pemphigold serum gave positive results; only faint and focal staining of the dermoepidermal junction was observed with bullous pemphigold serum. "

Therapies and Procedures

1.	Photopheresis 11/01/2012 - "Long-term efficacy of extracorporeal photochemotherapy in a patient with refractory epidermolysis bullosa acquisita." 09/01/1995 - "Remission of severe epidermolysis bullosa acquisita induced by extracorporeal photochemotherapy." 03/01/2015 - "We present a 12-year-old girl with refractory epidermolysis bullosa acquisita who responded to extracorporeal photopheresis. " 03/01/2015 - "The successful use of extracorporeal photopheresis in a 12-year-old patient with refractory epidermolysis bullosa acquisita." 03/01/1997 - "Treatment of refractory epidermolysis bullosa acquisita with extracorporeal photochemotherapy."
2.	Lasers (Laser) 05/01/2007 - "Two patients with localized epidermolysis bullosa acquisita: diagnostic value of laser scanning confocal microscopy." 04/01/1998 - "Application of confocal laser scanning microscopy to differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita." 08/01/2003 - "Laser scanning confocal microscopy allows precise localization of in vivo-bound IgG in patients' skin and, thus, it is a rapid method for the differentiation of mucous membrane pemphigoid from bullous pemphigoid and epidermolysis bullosa acquisita. " 04/01/1998 - "We applied confocal laser scanning microscopy to fluorescence overlay antigen mapping (FOAM) for differential diagnosis of bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA). " 03/01/2013 - "Fluorescence overlay antigen mapping using laser scanning confocal microscopy differentiates linear IgA bullous dermatosis from epidermolysis bullosa acquisita mediated by IgA."
3.	Tracheostomy 11/01/1993 - "The case is reported of a 56-year-old man with epidermolysis bullosa acquisita (EBA) who developed such severe involvement of the upper respiratory tract that a tracheostomy had to be performed. " 09/01/2001 - "Laryngeal epidermolysis bullosa acquisita requiring tracheostomy presenting with an acquired factor VIII coagulopathy." 11/01/1993 - "Epidermolysis bullosa acquisita--a case with upper airways obstruction requiring tracheostomy and responding to cyclosporin."
4.	Plasma Exchange 05/01/1986 - "Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita." 05/01/1986 - "Epidermolysis bullosa acquisita: clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer." 02/01/1997 - "We describe a case of severe epidermolysis bullosa acquisita of 7 years' duration that had been treated with azathioprine, corticosteroids, chlorambucil, plasma exchanges, cyclophosphamide, cyclosporine, and colchicine without any lasting effect. "
5.	Immunomodulation 12/01/2008 - "Subcutaneous immunoglobulin therapy for immunomodulation in a patient with severe epidermolysis bullosa acquisita."