Although epidermolysis bullosa acquisita (EBA) can be seen at any age, it has an increased prevalence in adulthood. The presentation of EBA is varied, with five clinical patterns being described so far.

Patients with classical EBA show skin fragility and the formation of non-inflammatory tense bullae that are prone to rupture. These lesions develop more on sites subject to minor trauma, such as the back of the hands, knuckles, elbows, knees and feet. The blisters heal with scarring and the formation of small inclusion cysts in the epidermis, termed milia [1]. Mucosal involvement is noted.

Bullous pemphigoid-like EBA shares clinical features with bullous pemphigoid, with patients presenting with lesions that are widespread, occurring in the trunk, extremities and skin folds [2]. Being surrounded by inflamed skin, the blisters are usually tense, with pruritus a common finding. Milia and scarring are not prominent.

A mucous membrane variant, termed cicatricial pemphigoid-like EBA, manifests with erosions and scarring on mucosal surfaces such as the mouth, conjunctiva, upper esophagus, vagina and anus [3]. The features of classical EBA may be absent in such cases.

The Brunsting-Perry pemphigoid-like variant of EBA occurs with bullae primarily on the head and neck region. Lesions often tend to heal with scarring with the involvement of mucosal lining being uncommon [4].

Immunoglobulin (Ig) A bullous dermatosis-like EBA appears with an annular arrangement of bullae seen characteristically in linear IgA bullous dermatosis [5]. Mucous membranes can be affected.

EBA rarely shows up in children, with the mucous membranes being more frequently involved.

EBA can occur concurrently with a variety of systemic diseases, such as systemic lupus erythematosus (SLE), inflammatory bowel disease (IBD), rheumatoid arthritis, amyloidosis, thyroiditis, leukemia, diabetes etc.

• Hemophilia A

  The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. [ncbi.nlm.nih.gov]

  Zillikens D. 2016 49 Myeloid-related proteins-8 and -14 are expressed but dispensable in the pathogenesis of experimental epidermolysis bullosa acquisita and bullous pemphigoid. ( 26692467 ) Akbarzadeh R....Petersen F. 2016 50 Coexistence of acquired hemophilia [malacards.org]

• Dysphagia

  Chua S, Dodd H, Saeed IT, et al. (2002) Dysphagia in a patient with lupus and review of the literature. Lupus 11: 322-324. xxiii.Kettler AH, Bean SF, Duffy JO, et al. (1988) Systemic lupus erythematosus presenting as a bullous eruption in a child. [kosmetischemedizin-online.de]

  It is suggested, however, that the dentist refer the patient to an ENT or GI specialist to evaluate dysphagia rather than empirically prescribe steroids without consulting a specialist. [cdeworld.com]

  One possible approach is to use phenytoin and oral steroid elixirs to reduce the symptoms of dysphagia, although generally steroids are avoided. If there is oral candidiasis, appropriate antifungal medication is helpful. [patient.info]

  In a subset of patients with predominant mucous membrane involvement, the disease manifests with blisters and scar formation in the oral, ocular, vaginal, and other mucous membranes, leading to significant dysfunction, such as visual function loss, dysphagia [emedicine.medscape.com]

  She complained of dysphagia due to involvement of the tongue and buccal mucosae. The blisters appeared spontaneously, and were not triggered by mechanical trauma. She had no itching, fever, or malaise. [jamanetwork.com]

• Diarrhea

  She was first put on colchicine, but as she had nausea and diarrhea at doses above 0.6 mg/day and was without any response, methotrexate was started. [ijdvl.com]

  Treatment includes regular and routine endoscopy and symptomatic treatment for hypogeusia and diarrhea. [clevelandclinicmeded.com]

• Nausea

  She was first put on colchicine, but as she had nausea and diarrhea at doses above 0.6 mg/day and was without any response, methotrexate was started. [ijdvl.com]

• Blister

  Epidermolysis Bullosa Acquisita definition Form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. [dermis.net]

  The blisters heal with scarring and the formation of small inclusion cysts in the epidermis, termed milia. Mucosal involvement is noted. [symptoma.com]

  Immunofluorescence on salt-split skin in epidermolysis bullosa acquisita reveals IgG in the blister floor (dermal side of split skin), while, in patients with bullous pemphigoid the IgG localises in the blister roof (epidermal side of split skin) Management [pcds.org.uk]

  We describe a 73-year-old Japanese man presenting with multiple, annular, tense blisters on the lower legs and oral lesions. [ncbi.nlm.nih.gov]

• Skin Disease

  Broad serologic screening is recommended unless a specific immunobullous skin disease type is suspected. [arupconsult.com]

  Author information 1 Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, D-23538 Lübeck, Germany. [email protected] Abstract Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering skin disease characterized by autoantibodies [ncbi.nlm.nih.gov]

  Rarely, people with lupus, a systemic autoimmune disease, develop a generalized blistering skin disease with the features of EBA. [rarediseases.info.nih.gov]

• Alopecia

  The association of EBA with other autoimmune diseases is common, but EBA occurring during alopecia areata has not been described previously. [ncbi.nlm.nih.gov]

  Nail dystrophy and scarring alopecia may also be present. [dermnet.com]

  Some patients have dystrophic nails and scarring alopecia, mucosal involvement, or ocular lesions leading to blindness. [merckmanuals.com]

• Skin Lesion

  (a-c) Skin lesions on the back at the first visit. (d) Aggravated skin lesions at the onset of MRSA sepsis, and (e) at remission after 2 cycles of IVIG. [omicsonline.org]

  The inflammatory form was recognised more recently and resembles bullous pemphigoid (see this term) with bullae developing on erythematous skin lesions, plaques without bullous eruptions and diffuse lesions that are not limited to trauma-prone sites. [orpha.net]

  The skin lesions responded to systemic immunosuppressant therapy and have regressed after allogeneic bone marrow transplantation. [ncbi.nlm.nih.gov]

• Dermatitis

  Consequently, dermatologists should be aware of this rare differential diagnosis of eyelid dermatitis where a contact allergy or atopic dermatitis is absent. [ncbi.nlm.nih.gov]

  Transient acantholytic dermatosis Pemphigoid ( dermis ) IgG : Bullous pemphigoid Cicatricial pemphigoid Localised Gestational pemphigoid Pemphigoid nodularis Epidermolysis bullosa acquisita IgA : Linear IgA bullous dermatosis Childhood Adult Other bullous Dermatitis [en.wikipedia.org]

  […] herpetiformis Contact dermatitis Drug eruption Bullous SLE Hereditary forms of EBA Monitoring Either collagen type VII IgG antibody and epithelial skin antibody or collagen type VII IgG antibody and IgG BMZ antibodies Background Epidemiology Incidence [arupconsult.com]

  수포증, 상세불명의 표피 수포증 Polish Oddzielanie się naskórka pęcherzowe Hungarian Epidermolysis bullosa Norwegian Epidermolysis bullosa Croatian Epidermolysis bullosa, Bulozna epidermoliza Spanish Acantólisis Bulosa, Epidermólisis Bullosa, acantólisis bullosa, dermatitis [fpnotebook.com]

EBA shares many clinical and immune-pathological features with other subepithelial blistering disorders and so its identification may hence be challenging. A thorough patient history and full skin examination help to narrow the differential diagnosis. Additional tests must be performed to confirm the disease.

The histopathological findings vary with the clinical subtype, with early lesions showing blister formation along the dermal-epithelial junction (DEJ) with adjacent papillary edema [6]. The intensity of inflammation may vary with the type of lesion encountered.

Direct immunofluorescence shows the deposition of immunoglobulins in almost all cases. Classical EBA presents with the deposition of IgG and complement along the DEJ. Some subtypes may also showcase the presence of IgA, IgM, factor B and properdin [7].

Indirect immunofluorescence using the salt-split skin technique may demonstrate the presence of antibody binding to the dermal side of the basement membrane. This test may help to differentiate EBA from other autoimmune disorders causing subepithelial blister formation [8].

Additional studies may be done to support a diagnosis of EBA in the setting of a negative immunofluorescence report. These include direct and indirect immunoelectron microscopy, enzyme-linked immunosorbent assay (ELISA), immunoblotting, immunoprecipitation and collagen immunomapping techniques [7] [9].

Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications. Copyright © 2012 Elsevier España, S.L. and AEDV. [ncbi.nlm.nih.gov]

Thus, IVIG was used for the treatment for sepsis, resulting in improvements of both sepsis and mucocutaneous lesions. Several lines of evidence indicate that IVIG is effective in the treatment of EBA. [omicsonline.org]

Prognosis EBA is a chronic disease that resolves slowly and leads to dystrophic scarring and milia. [orpha.net]

Although typically associated with a good prognosis in children, there are rare cases that are refractory to treatment with conventional immunosuppressive therapy. [ncbi.nlm.nih.gov]

Corticosteroids and dapsone Skin care The prognosis is variable, but disease course tends to be prolonged. High-quality evidence about treatments is lacking, and treatment recommendations are often anecdotal. [merckmanuals.com]

Although the precise etiology of EBA is unknown, most of the evidence suggests an autoimmune etiology. [accessmedicine.mhmedical.com]

The etiology of epidermolysis bullosa acquisita (EBA) is unknown. [ncbi.nlm.nih.gov]

Definition / general Rare, non-congenital, autoimmune, chronic listering disease of skin and mucus membranes ( eMedicine ) Etiology Usually IgG autoantibodies against NC1 (noncollagenous domain of type VII collagen), major component of anchoring fibrils [pathologyoutlines.com]

Etiology EBA is caused by the production of antibodies against the skin basement membrane collagen VII, the major component of the anchoring fibrils located in the dermal-epidermal junction, under the lamina densa. [orpha.net]

Summary Epidemiology The prevalence is unknown but the incidence is estimated at 1 in 96,200 new cases per year. Clinical description The disease manifests in two clinical forms: a classical form and an inflammatory form. [orpha.net]

[…] cutanea tarda Dermatitis herpetiformis Contact dermatitis Drug eruption Bullous SLE Hereditary forms of EBA Monitoring Either collagen type VII IgG antibody and epithelial skin antibody or collagen type VII IgG antibody and IgG BMZ antibodies Background Epidemiology [arupconsult.com]

Extramammary Paget Disease: Epidemiology and Association to Cancer in a Quebec-Based Population. J Low Genit Tract Dis. 2013 Apr;17(2):167-74. 5. Lam C, Vleugels RA. Management of Cutaneous Dermatomyositis. [bumc.bu.edu]

References Horner ME, Alikhan A, Tintle S, Tortorelli S, Davis DM, Hand JL: Cutaneous porphyrias part I: epidemiology, pathogenesis, presentation, diagnosis, and histopathology. Int J Dermatol 2013;52:1464-1480. Elder GH: Porphyria cutanea tarda. [karger.com]

Despite recent progress in understanding the pathophysiology of EBA, its diagnosis is still challenging. Areas covered: This review provides an update on the clinical manifestations and diagnostic methods of EBA. [ncbi.nlm.nih.gov]

Useful For Suggests clinical disorders or settings where the test may be helpful Confirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus Clinical Information Discusses physiology, pathophysiology [mayomedicallaboratories.com]

SLE Hereditary forms of EBA Monitoring Either collagen type VII IgG antibody and epithelial skin antibody or collagen type VII IgG antibody and IgG BMZ antibodies Background Epidemiology Incidence – 25/100,000 Age – peak onset in 40s Sex – M:F, equal Pathophysiology [arupconsult.com]

This new edition of Essential Clinical Immunology is fully updated, now in full colour throughout and features: coverage of the underlying pathophysiology and signs and symptoms of disease, as well as the special investigations required and management [books.google.com]

2017 42 Long-term results of rituximab-intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy. ( 27161164 ) Oktem A...Kocyigit P 2017 43 Epidermolysis Bullosa Acquisita: From Pathophysiology [malacards.org]

Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [icdlist.com]

Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications. Copyright © 2012 Elsevier España, S.L. and AEDV. [ncbi.nlm.nih.gov]

The timely identification of SIDM, when not preventable, is therefore crucial so that cardiovascular risk factors can be optimized and good glycemic control can be achieved early in the patient’s course. [cureus.com]

Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications. [actasdermo.org]

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3. Dahl MG. Epidermolysis bullosa acquisita – a sign of cicatricial pemphigoid? Br J Dermatol. 1979;101:475–483.
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6. Elston DM, Stratman EJ, Miller SJ. Skin biopsy: Biopsy issues in specific diseases. J Am Acad Dermatol. 2016 Jan;74 (1):1-16; quiz 17-8.
7. Lehman JS, Camilleri MJ, Gibson LE. Epidermolysis bullosa acquisita: concise review and practical considerations. Int J Dermatol. 2009 Mar;48(3):227-35; quiz 235-6
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9. Vodegel RM, de Jong MC, Pas HH, Jonkman MF. IgA-mediated epidermolysis bullosa acquisita: Two cases and review of the literature. J Am Acad Dermatol. 2002;6:919–25.