A 20-year-old woman diagnosed as having epidermolysis bullosa acquisita (EBA) presented
with dysphagia and odynophagia. Upper gastrointestinal endoscopy revealed a web in
the upper esophagus, for which endoscopic dilatation was done. Following this, the
patient remained asymptomatic for 2 years, after which she again presented with recurrence
of symptoms. Clinical examination showed multiple erythematous and maculopapular skin
eruptions, predominantly on the face and nape of the neck ([Fig. 1]). Laboratory investigation revealed increased total white cell count, hemoglobin
10 g/dL, and a raised erythrocyte sedimentation rate (ESR). Despite the patient being
on treatment with steroids for her primary disease, she had an acute exacerbation
with severe dysphagia and odynophagia. A barium swallow showed a tight stricture in
a small segment of the upper esophagus ([Fig. 2]). Upper gastrointestinal endoscopy ([Fig. 3]) also showed the tight stricture in the upper esophagus, with friable, edematous
mucosa. The endoscope could not be passed beyond the stricture, and the patient underwent
careful endoscopic balloon dilation ([Fig. 4]) under general anesthesia. She recovered well postoperatively and was gradually
started on feeds, which she was able to swallow comfortably.
Fig. 1 An erythematous maculopapular lesion in a young woman with epidermolysis bullosa,
presenting with dysphagia and odynophagia.
Fig. 2 Barium swallow showing a tight stricture in the upper esophagus.
Fig. 3 Endoscopy showing mucosal edema, ulceration, and narrowing in upper esophagus.
Fig. 4 Endoscopic appearance following esophageal balloon dilation.
Epidermolysis bullosa involves not only the skin but also the internal organs that
are lined with squamous epithelium. Clinically, the disease presents in three main
forms, with mild inflammatory form of EBA being the commonest. Mucosal involvement
can lead to complications such as esophageal strictures causing dysphagia [1]
[2]
[3]. Intensive nutritional support followed by balloon dilation is the first choice
of treatment for esophageal strictures complicating epidermolysis bullosa. By following
this regimen, invasive surgery can be avoided [4]. There is no satisfactory medical treatment of dysphagia, and endoscopic balloon
dilation has been found to be a safe and effective approach [5]. However, caution has to be exercised during this procedure due to the likelihood
of complications such as bullae formation, bleeding, and perforation at the dilation
site.
Endoscopy_UCTN_Code_CCL_1AB_2AC_3AD
