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Laryngeal Manifestations of Epidermolysis Bullosa Acquisita FREE

Jerome E. Hester, MD; David P. Arnstein, MD; David Woodley, MD
Arch Otolaryngol Head Neck Surg. 1995;121(9):1042-1044. doi:10.1001/archotol.1995.01890090078015
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Epidermolysis bullosa acquisita is an acquired blistering skin disease caused by autoantibodies to the dermoepidermal junction of the skin. Patients present with tense bullae over areas of trauma, commonly the palms, soles, elbows, and knees. When the lesions heal, they leave a dense scar. In the head and neck, bullae in the buccal mucosa and the cervical esophagus cause strictures. To our knowledge, this is the first reported case of epidermolysis bullosa acquisita of the larynx. The patient had severe supraglottic stenosis that was caused by the trauma of a routine intubation. We describe the clinical and histologic characteristics of the disease and suggest managing the strictures with balloon dilation to minimize trauma and prevent further lesions and scarring.

(Arch Otolaryngol Head Neck Surg. 1995;121:1042-1044)

REFERENCES

Sedano HO, Gorlin DJ.  Epidermolysis bullosa . Oral Surg Oral Med Oral Pathol . 1989;;67:555-563.
Stewart MI, Woodley DT, Briggaman RA.  Epidermolysis bullosa acquisita and associated symptomatic esophageal webs . Arch Dermatol . 1991;; 127:373-377.
Woodley DT, Burgeson RE, Lunstrom G, Bruckerner-Tuderman L, Reese MJ, Briggaman RA.  Epidermolysis bullosa acquisita antigen is the globular carboxyl terminus type VII procollagen . J Clin Invest . 1988;;8:683-687.
Woodley DT, Briggaman RA, O'Keefe EJ, Inman AO, Queen LL, Gammon WR.  Identification of the skin basement membrane autoantigen in epidermolysis bullosa acquisita . N Engl J Med .1984;; 310:1007-1013.
Woodley DT, Briggaman RA, Gammon WR.  Acquired epidermolysis bullosa . Dermatol Clin .1990;: 8:717-726.
Prost C, Labeille B, Chaussade V, Guillaume JC, Martin N, Dubertret L.  Immunoelectron microscopy in subepidermal autoimmune bullous diseases: a prospective study of IgG and C3 bound in vivo in 32 patients . J Invest Dermatol . 1987;; 89:567-573.
Briggaman RA, Gammon WR, Woodley DT.  Epidermolysis bullosa acquisita of the immunopatholical type . J Invest Dermatol . 1985;;85( (suppl) ): 79-84.
Gammon WR, Briggaman RA, Woodley DT, Heald DW, Wheeler CE.  Epidermolysis bullosa acquisita: a pemphigoid like disease . J Am Acad Dermatol . 1984;;11:820-832.
Berson S, Ward RF, Lin AN, Carter DM.  Junctional epidermolysis bullosa of the larynx: report of a case and literature review . Ann Otol Rhinol Laryngol . 1992;;101:861-865.
Hellman D, Laing T, Petri M, Jacobs D, Crumley R, Stulbarg M.  Wegener's granulomatosus: isolated involvement of the trachea and larynx . Ann Rheum Dis . 1987;;46:628-631.
Mills RP, O'Connor MJ, Shun-Shin GA:  Mucous membrane pemphigoid: a preventable cause of laryngeal stenosis . J Laryngol Otol . 1983;;97: 761-765.
Jafek BW, Esses BA.  Manifestations of systemic diseases . Otolaryngology—Head and Neck Surgery . St Louis, Mo: CV Mosby Co; 1986;: chap 102.
Crow LL, Finkle JP, Gammon WR, Woodley DT.  Clearing of EBA on cyclosporine A . J Am Acad Dermatol . 1988;;19:937-942.
Layton AM.  Clearing of EBA with cyclosporine . JAm Acad Dermatol . 1990;;22:535-539.

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Sedano HO, Gorlin DJ.  Epidermolysis bullosa . Oral Surg Oral Med Oral Pathol . 1989;;67:555-563.
Stewart MI, Woodley DT, Briggaman RA.  Epidermolysis bullosa acquisita and associated symptomatic esophageal webs . Arch Dermatol . 1991;; 127:373-377.
Woodley DT, Burgeson RE, Lunstrom G, Bruckerner-Tuderman L, Reese MJ, Briggaman RA.  Epidermolysis bullosa acquisita antigen is the globular carboxyl terminus type VII procollagen . J Clin Invest . 1988;;8:683-687.
Woodley DT, Briggaman RA, O'Keefe EJ, Inman AO, Queen LL, Gammon WR.  Identification of the skin basement membrane autoantigen in epidermolysis bullosa acquisita . N Engl J Med .1984;; 310:1007-1013.
Woodley DT, Briggaman RA, Gammon WR.  Acquired epidermolysis bullosa . Dermatol Clin .1990;: 8:717-726.
Prost C, Labeille B, Chaussade V, Guillaume JC, Martin N, Dubertret L.  Immunoelectron microscopy in subepidermal autoimmune bullous diseases: a prospective study of IgG and C3 bound in vivo in 32 patients . J Invest Dermatol . 1987;; 89:567-573.
Briggaman RA, Gammon WR, Woodley DT.  Epidermolysis bullosa acquisita of the immunopatholical type . J Invest Dermatol . 1985;;85( (suppl) ): 79-84.
Gammon WR, Briggaman RA, Woodley DT, Heald DW, Wheeler CE.  Epidermolysis bullosa acquisita: a pemphigoid like disease . J Am Acad Dermatol . 1984;;11:820-832.
Berson S, Ward RF, Lin AN, Carter DM.  Junctional epidermolysis bullosa of the larynx: report of a case and literature review . Ann Otol Rhinol Laryngol . 1992;;101:861-865.
Hellman D, Laing T, Petri M, Jacobs D, Crumley R, Stulbarg M.  Wegener's granulomatosus: isolated involvement of the trachea and larynx . Ann Rheum Dis . 1987;;46:628-631.
Mills RP, O'Connor MJ, Shun-Shin GA:  Mucous membrane pemphigoid: a preventable cause of laryngeal stenosis . J Laryngol Otol . 1983;;97: 761-765.
Jafek BW, Esses BA.  Manifestations of systemic diseases . Otolaryngology—Head and Neck Surgery . St Louis, Mo: CV Mosby Co; 1986;: chap 102.
Crow LL, Finkle JP, Gammon WR, Woodley DT.  Clearing of EBA on cyclosporine A . J Am Acad Dermatol . 1988;;19:937-942.
Layton AM.  Clearing of EBA with cyclosporine . JAm Acad Dermatol . 1990;;22:535-539.

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