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.

• Nasal Congestion

  She referred progressive association of bilateral eyelid retraction with epifora, nasal congestion with saddle nose deformity and recurrent episodes of skin eruption associated with trauma over the knuckles, elbows and extensor surfaces of the arms, unresponsive [academic.oup.com]

• Saddle Nose

  She referred progressive association of bilateral eyelid retraction with epifora, nasal congestion with saddle nose deformity and recurrent episodes of skin eruption associated with trauma over the knuckles, elbows and extensor surfaces of the arms, unresponsive [academic.oup.com]

• 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]

  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]

  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]

  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]

• Blister

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

  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]

• Skin Lesion

  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]

  lesion removal (Medical Encyclopedia) Skin lesion removal-aftercare (Medical Encyclopedia) Stasis dermatitis and ulcers (Medical Encyclopedia) [ Read More ] [icdlist.com]

• Nikolsky's Sign

  The development of a blister following self induced trauma, the positive Nikolsky's sign, bulla spreading sign and a subepidermal bulla were in favour of a diagnosis of epidermolysis bullosa acquisita. [ijdvl.com]

  Nikolsky’s sign was positive. In addition, mucosal lesions appeared on the tongue and lips. [omicsonline.org]

  The Nikolsky and Asboe-Hansen signs (see above) are positive. The crusted lesions may follow an arcuate pattern. Healing occurs without scarring. [plasticsurgerykey.com]

  The Nikolsky sign was negative. The results of an examination of the mucous membranes revealed red conjunctivae; erosions with crusts in the antrum nasi; and vesicles on the palate, buccal mucosa, and tongue and in the vagina. Figure 1. [jamanetwork.com]

• Cutaneous Manifestation

  Figure 1: Cutaneous manifestations. (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]

  Abstract Porphyria cutanea tarda (PCT) is the most common type of porphyria worldwide and is often initially diagnosed when cutaneous manifestations arise. [karger.com]

  Box 1 outlines the most common cutaneous manifestations of diabetes, arranged by frequency of occurrence (most to least frequent). [clevelandclinicmeded.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]

Management and treatment The first line treatment revolves around administration of dapsone or sulfasalazine. Immunosuppresive therapy (such as treatment with cyclosporine) may by required in severe cases. [orpha.net]

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]

Prognosis - Epidermolysis bullosa acquisita Not supplied. Treatment - Epidermolysis bullosa acquisita Not supplied. Resources - Epidermolysis bullosa acquisita Not supplied. [checkorphan.org]

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]

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]

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]

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]

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]

As knowledge about the pathophysiology of EB has become more advanced, new variants of EB have been identified based on their specific genetic mutation. [ 1 ] Investigations [ 11 ] Skin biopsy is required. [patient.info]

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 aim of treatment is to manage the disease to prevent long-term problems from scarring. [dermcoll.edu.au]

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]

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]

1. Harman KE, Whittam LR, Wakelin SH, et al. Severe, refractory epidermolysis bullosa acquisita complicated by an oesophageal stricture responding to intravenous immune globulin. Br J Dermatol. 1998;139:1126–1127.
2. Gammon WR, Briggaman RA, Woodley DT, et al. Epidermolysis bullosa acquisita – a pemphigoid like disease. J Am Acad Dermatol. 1984:820–832.
3. Dahl MG. Epidermolysis bullosa acquisita – a sign of cicatricial pemphigoid? Br J Dermatol. 1979;101:475–483.
4. Kurzhals G, Stolz W, Meurer M, et al. Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid. Arch Dermatol. 1991;127:391–395.
5. Callot-Mellot C, Bodemer C, Caux F, et al. Epidermolysis bullosa aquisita in childhood. Arch of Dermatol. 1997;133:1122–1126.
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
8. Woodley D, Remington J, Chen M. Autoimmunity to type VII Collagen: Epidermolysis Bullosa Acquisita. Clin Rev Allerg Immunol. 2007;33:78–84.
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.