Laryngeal Manifestations of Epidermolysis Bullosa Acquisita

Hester, Jerome E.; Arnstein, David P.; Woodley, David T.

doi:10.1001/archotol.1995.01890090078015

Cited by 16 publications

(13 citation statements)

References 12 publications

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“…The articles identified from our search included 63 case reports7–58 and 10 case series,24, 59–66 representing 141 patients with laryngeal involvement of MMP. No clinical trials or comparative trials of laryngeal MMP were found.…”

Section: Resultsmentioning

confidence: 99%

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Laryngeal mucous membrane pemphigoid: A systematic review and pooled‐data analysis

2010

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Section: Resultsmentioning

confidence: 99%

“…The role of surgery in this disease remains unclear. Several authors report anecdotal success with bougie or balloon dilatation, laser excision, and laryngotracheal resections 25. There is concern that traumatizing the mucosa may actually worsen the scarring and lead to further stenosis.…”

Section: Discussionmentioning

confidence: 99%

Laryngeal mucous membrane pemphigoid: A systematic review and pooled‐data analysis

2010

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“…Rarely, laryngeal involvement may cause hoarseness, impaired phonation, and loss of voice, and may lead to irreversible respiratory distress. These may be caused by trauma induced by intubation [24, 25]. …”

Section: Extracutaneous Eba Manifestations and Associated Diseasesmentioning

confidence: 99%

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Ludwig

2013

ISRN Dermatology

124

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Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous autoimmune skin blistering disease. The pathogenic relevance of autoantibodies targeting type VII collagen (COL7) has been well-documented. Therefore, EBA is a prototypical autoimmune disease with a well-characterized pathogenic relevance of autoantibody binding to the target antigen. EBA is a rare disease with an incidence of 0.2 new cases per million and per year. The current treatment of EBA relies on general immunosuppressive therapy, which does not lead to remission in all cases. Therefore, there is a high, so far unmet medical need for the development of novel therapeutic options. During the last 10 years, several novel in vitro and in vivo models of EBA have been established. These models demonstrated a critical role of the genetic background, T cells, and cytokines for mediating the loss of tolerance towards COL7. Neutrophils, complement activation, Fc gamma receptor engagement, cytokines, several molecules involved in cell signaling, release of reactive oxygen species, and matrix metalloproteinases are crucial for autoantibody-induced tissue injury in EBA. Based on this growing understanding of the diseases' pathogenesis, several potential novel therapeutic targets have emerged. In this review, the clinical presentation, pathogenesis, diagnosis, and current treatment options for EBA are discussed in detail.

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“…The second case [18]responded well to treatment with cyclosporine. In a third case [19], surgical management of these lesions with balloon dilatation is discussed. To our knowledge, tracheal involvement has never been described before.…”

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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Laryngeal Manifestations of Epidermolysis Bullosa Acquisita

Cited by 16 publications

References 12 publications

Laryngeal mucous membrane pemphigoid: A systematic review and pooled‐data analysis

Laryngeal mucous membrane pemphigoid: A systematic review and pooled‐data analysis

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

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

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