AJR 2003; 180:749-750
© American Roentgen Ray Society
Diffuse Esophageal Stricture Caused by Erythema Multiforme Major
Laura R. Carucci1,
Marc S. Levine and
Stephen E. Rubesin
1 All authors: Department of Radiology, Hospital of the University of
Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104.
Received May 24, 2002;
accepted after revision June 5, 2002.
Address correspondence to M. S. Levine.
Introduction
Erythema multiforme is a hypersensitivity reaction triggered by various
stimuli or by reactivation of a latent infection
[1,2,3].
This condition is characterized by a maculopapular or bullous rash, with the
lesions having a classic target appearance
[1,
2]. Although erythema
multiforme occurs at any age, it usually is a disease of the first three
decades of life
[1,2,3].
The disorder may be classified as erythema multiforme minor when it is
confined to the skin and as erythema multiforme major when it also involves
mucous membranes of the eyes, oropharynx, genitalia, or anus, and, rarely, the
tracheobronchial tree or esophagus
[1,
2]. Erythema multiforme major
is a potentially serious condition, with mortality rates ranging from 3% to
25%
[2,3,4].
Stevens-Johnson syndrome is a life-threatening form of esophageal multiforme
major with associated constitutional symptoms
[2].
Esophageal involvement by erythema multiforme major usually is
self-limited, but occasionally children or adolescents have been reported with
dysphagia caused by esophageal strictures, including five patients with focal
strictures in the upper or mid esophagus, one with a focal stricture in the
distal esophagus, and one (a 7-year-old child) with a diffuse esophageal
stricture
[2,3,4,5,6,7,8].
We recently encountered an adult patient with erythema multiforme major who
presented with dysphagia caused by a diffuse esophageal stricture. To our
knowledge, such strictures have not been described previously in adults with
this disorder.
Case Report
A 48-year-old woman presented with a 7-month history of dysphagia for
solids. She previously had suffered from a recurrent rash. Biopsy specimens
from the skin lesions revealed findings of erythema multiforme. She also had a
chronic history of conjunctivitis, sinusitis, vaginitis, and
tracheobronchitis, and had experienced two episodes of acute airway
obstruction from sloughing of tracheal mucosa. Laboratory tests for
antimitochondrial antibody, antinuclear antibody, anti—smooth muscle
antibody, anticardiolipin antibody, rheumatoid factor, HIV, and infectious
conditions were all negative. Therefore, a diagnosis was made of erythema
multiforme major, and the patient was treated intermittently with antibiotics
and steroids.
A double-contrast esophagogram with high-density barium revealed a diffuse
stricture with a smooth contour and tapered borders that involved the entire
thoracic esophagus (Fig.
1A,1B).
Subsequent endoscopy revealed erosive esophagitis with a long stricture
containing erythematous friable mucosa. Endoscopic biopsy specimens revealed
inflammatory changes as well as separation of the mucosa from the submucosa,
histopathologic findings compatible with erythema multiforme major involving
the esophagus. Immunofluorescent stains were negative for bullous
pemphigoid.
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Fig. 1A. —48-year-old woman with diffuse esophageal stricture caused by
erythema multiforme major. Left posterior oblique view from double-contrast
esophagogram with high-density barium shows diffuse stricture involving
thoracic esophagus. Note that stricture begins proximally at level of
clavicles (arrow).
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Fig. 1B. —48-year-old woman with diffuse esophageal stricture caused by
erythema multiforme major. Another left posterior oblique view centered lower
than A shows how stricture extends distally to just above
gastroesophageal junction (arrow).
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Discussion
To our knowledge, no adults have been reported with a diffuse esophageal
stricture caused by erythema multiforme major. Other more common causes of
diffuse esophageal strictures include caustic ingestion, mediastinal
irradiation, and nasogastric intubation. However, esophageal involvement by
erythema multiforme major should be suspected in patients with characteristic
mucocutaneous lesions. Epidermolysis bullosa dystrophica, bullous pemphigoid,
and benign mucous membrane pemphigoid are other rare dermatologic disorders
associated with the development of bullous lesions on the skin and esophageal
strictures, but patients with these disorders usually have focal strictures or
webs involving the cervical or upper thoracic esophagus and characteristic
histopathologic or immunofluorescent findings. Thus, in the appropriate
clinical setting, erythema multiforme major should be included in the
differential diagnosis of a diffuse stricture involving the esophagus in
adults.
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