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 . 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 . 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 . 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 .
Immunoglobulin (Ig) A bullous dermatosis-like EBA appears with an annular arrangement of bullae seen characteristically in linear IgA bullous dermatosis . 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.
Entire Body System
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]
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]
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]
Here we identify Flii as a novel target involved in skin blistering. [ncbi.nlm.nih.gov]
- Skin Disease
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]
Broad serologic screening is recommended unless a specific immunobullous skin disease type is suspected. [arupconsult.com]
- Skin Lesion
In the classical form, onset occurs in adulthood and the bullae may be soft, tense or haemorrhagic, located on otherwise healthy skin. Lesions are usually triggered by minor trauma and are mainly localised to sites that are easily injured. [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]
A blister can be provoked by tangential pressure on normal-appearing skin adjacent to a lesion (Nikolsky sign). The involved areas heal 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 . 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 .
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 .
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  .
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