The spectrum of epidermolysis bullosa acquisita

Richter, B. J.

doi:10.1001/archderm.115.11.1325

Cited by 25 publications

(5 citation statements)

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“…However, a nonscarring (inflammatory) phase is seen in EBA, and only 1 case of nonscarring EBA has been reported [12]. Then, a few cases of EBA with esophageal involvement were described [13, 14, 15, 16]. Therefore, our case could be classified as EBA.…”

Section: Discussionmentioning

confidence: 91%

A Case of an Inflammatory Variant of Epidermolysis bullosa acquisita: Chronic Bullous Dermatosis Associated with Nonscarring Mucosal Blisters and Circulating IgG Anti-Type-VII-Collagen Antibody

et al. 1998

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A 42-year-old man showed prominent blistering lesions of the mouth and esophagus in addition to a few bullous lesions of the skin. Direct immunofluorescence microscopy revealed distinct linear deposition of IgG and C3 at the epidermal basement membrane zone where slight deposition of IgA and IgM was also observed. In direct immunoelectron-microscopic examination, antibody was detected in the sublamina densa of the basement membrane zone. Immunoblot analysis with dermal extracts demonstrated that the patient’s serum contained circulating IgG antibodies against the 290-kD protein, which comigrated with type VII collagen. The lesions healed without any scars. The results of these studies corresponded to the laboratory findings in epidermolysis bullosa acquisita (EBA), although the clinical features were distinct from classic EBA.

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Section: Discussionmentioning

confidence: 91%

A Case of an Inflammatory Variant of Epidermolysis bullosa acquisita: Chronic Bullous Dermatosis Associated with Nonscarring Mucosal Blisters and Circulating IgG Anti-Type-VII-Collagen Antibody

et al. 1998

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“…Ultrastructural studies of EBA skin have demonstrated a paucity of AFs and an amorphous, electron-dense band just beneath the lamina densa [50, 51]. Although the autoantibodies are directed against the AFs in the sublamina densa region of the BMZ, it should be noted that the cleavage plane of the blister may be either in the lamina lucida or the sublamina densa region where AFs are located [52].…”

Section: Laboratory Testsmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

146

112

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EBA is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen structures, a major component of anchoring fibrils, that attach the epidermis onto the dermis. EBA patients have tissue-bound as well as circulating anti-type VII collagen autoantibodies that attack type VII collagen and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss: all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-type VII collagen antibodies are “pathogenic” because when injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmaphoresis, photopheresis, infliximab, and intravenous immunoglobulin.

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“…EBA-induced laryngeal stenosis requiring tracheotomy has been reported only three times in the literature. The first case was described by Richter and McNutt in 1979 [17]. However, it was not documented by the criteria now used for the diagnosis of EBA since immunofluorescence on saline-split skin and immunoblotting were not available at that time.…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

Wieme¹,

Lambert²,

Moerman

et al. 1999

Dermatology

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Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.

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The spectrum of epidermolysis bullosa acquisita

Cited by 25 publications

References 0 publications

A Case of an Inflammatory Variant of Epidermolysis bullosa acquisita: Chronic Bullous Dermatosis Associated with Nonscarring Mucosal Blisters and Circulating IgG Anti-Type-VII-Collagen Antibody

A Case of an Inflammatory Variant of Epidermolysis bullosa acquisita: Chronic Bullous Dermatosis Associated with Nonscarring Mucosal Blisters and Circulating IgG Anti-Type-VII-Collagen Antibody

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

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