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Bullous pemphigoid: extensive esophageal involvement
Accepted Manuscript Bullous pemphigoid: extensive esophageal involvement R. Gaspar, P. Moutinho-Ribeiro, G. Macedo PII:
S0016-5107(17)30117-7
DOI:
10.1016/j.gie.2017.02.015
Reference:
YMGE 10427
To appear in:
Gastrointestinal Endoscopy
Received Date: 18 December 2016 Accepted Date: 17 February 2017
Please cite this article as: Gaspar R, Moutinho-Ribeiro P, Macedo G, Bullous pemphigoid: extensive esophageal involvement, Gastrointestinal Endoscopy (2017), doi: 10.1016/j.gie.2017.02.015. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
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Bullous pemphigoid: extensive esophageal involvement
M AN U
Rui Gaspar1, Pedro Moutinho-Ribeiro1, Guilherme Macedo1 1
Gastroenterology Department, Centro Hospitalar São João, Porto, Portugal Porto Medical School, Portugal.
TE D
Porto WGO Training Center, Portugal.
EP
Corresponding author: Rui Gaspar M.D. Department of Gastroenterology, Faculty of Medicine, Centro
AC C
Hospitalar São João.
Alameda Prof. Hernani Monteiro 4200-319 Porto, Portugal. Tel.: +351 22 551 3600. Fax: +351 22 551 3601. E-mail address: [email protected]
ACCEPTED MANUSCRIPT Bullous pemphigoid: extensive esophageal involvement Gaspar R. , Moutinho-Ribeiro P. , Macedo G.1 1
1
Gastroenterology Department, Centro Hospitalar São João, Porto, Portugal Porto Medical School, Portugal.
RI PT
Porto WGO Training Center, Portugal.
A 73-year-old woman with a medical history of bullous pemphigoid diagnosed in 2013, who was being treated with prednisolone 20 mg daily, was admitted to the Internal Medicine Department with Escherichia coli urosepsis. She began treatment with ceftriaxone 2g/day, and the dose of oral prednisolone was lowered to 10 mg/daily.
M AN U
SC
One week later, due to worsening of the cutaneous lesions, she was seen by a dermatologist, who described extensive ulcerated cutaneous lesions, with no infection signs. This clinical deterioration led to the decision of increasing prednisolone to the pre-admission dosage. Due to a de novo bilateral pleural effusion with dyspnea as the only associated symptom, a CT scan was performed that revealed an incidental finding of a de novo distension of the middle third of the esophagus. Upper endoscopy was then performed, which showed extensive continuous and diffuse esophageal involvement from 20 to 35 cm from the incisors, with severe friability of the mucosa, bullous and necrotic areas, as well as segments with mucosa sloughing, self-limited oozing hemorrhage, and subepithelial hematomas.
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In this case, the reduction of the prednisolone dosage might have explained the clinical deterioration of the cutaneous lesions, as well as the exuberance of esophageal involvement.
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TE D
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Commentary on GIE-D-16-01738R1
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This case represents an uncommon condition, with a unique GI manifestation. Pemphigoid disorders, which encompass bullous pemphigoid and mucous membrane pemphigoid (MMP), are a spectrum of very rare autoimmune dermatologic conditions. A slight predominance in the elderly and female population has been noted. The pathogenesis involves circulating antibodies that are directed against the basement membrane of squamous epithelium, with subsequent complement activation and inflammatory infiltrate. In the case of bullous pemphigoid, tense bullae on skin surfaces are the consequence of this inflammatory cascade, whereas oral and conjunctival mucosal disease predominates in MMP.
M AN U
In 1978, Eng et al described the first case of extensive esophageal bullae causing upper gastrointestinal hemorrhage. In prior reports of MMP with esophageal involvement, which happens in 4% of cases, patients presented with chest pain, dysphagia, and odynophagia.
AC C
EP
TE D
Interestingly, dissociation between symptoms and endoscopy is evident in this case because the patient was entirely asymptomatic from a GI standpoint. EGD can be challenging in cases like this, due to bullae formation and mucosal sloughing after a light touch of the endoscope of the esophageal mucosa. Gentle movement of the endoscope is recommended in order to prevent adverse events such as bleeding, laceration, and perforation. Therapy is directed toward controlling the underlying immune disease. Systemic steroids are the therapy of choice to achieve remission, as described in this case. However, in cases with brisk GI bleeding causing hemodynamic instability, therapeutic endoscopy can be used to achieve hemostasis. Argon plasma coagulation, which does not require contact with the mucosa, has been successfully used in the past. This case encourages gastroenterologists to be aware of the digestive manifestations of dermatologic diseases and to cautiously perform endoscopy in patients with pemphigoid diseases, even in the absence of overt symptoms. Lady Katherine Mejía Pérez, MD Research Fellow Mayo Clinic Florida Massimo Raimondo, MD Associate Editor for Focal Points
S0016-5107(17)30117-7
DOI:
10.1016/j.gie.2017.02.015
Reference:
YMGE 10427
To appear in:
Gastrointestinal Endoscopy
Received Date: 18 December 2016 Accepted Date: 17 February 2017
Please cite this article as: Gaspar R, Moutinho-Ribeiro P, Macedo G, Bullous pemphigoid: extensive esophageal involvement, Gastrointestinal Endoscopy (2017), doi: 10.1016/j.gie.2017.02.015. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
SC
RI PT
Bullous pemphigoid: extensive esophageal involvement
M AN U
Rui Gaspar1, Pedro Moutinho-Ribeiro1, Guilherme Macedo1 1
Gastroenterology Department, Centro Hospitalar São João, Porto, Portugal Porto Medical School, Portugal.
TE D
Porto WGO Training Center, Portugal.
EP
Corresponding author: Rui Gaspar M.D. Department of Gastroenterology, Faculty of Medicine, Centro
AC C
Hospitalar São João.
Alameda Prof. Hernani Monteiro 4200-319 Porto, Portugal. Tel.: +351 22 551 3600. Fax: +351 22 551 3601. E-mail address: [email protected]
ACCEPTED MANUSCRIPT Bullous pemphigoid: extensive esophageal involvement Gaspar R. , Moutinho-Ribeiro P. , Macedo G.1 1
1
Gastroenterology Department, Centro Hospitalar São João, Porto, Portugal Porto Medical School, Portugal.
RI PT
Porto WGO Training Center, Portugal.
A 73-year-old woman with a medical history of bullous pemphigoid diagnosed in 2013, who was being treated with prednisolone 20 mg daily, was admitted to the Internal Medicine Department with Escherichia coli urosepsis. She began treatment with ceftriaxone 2g/day, and the dose of oral prednisolone was lowered to 10 mg/daily.
M AN U
SC
One week later, due to worsening of the cutaneous lesions, she was seen by a dermatologist, who described extensive ulcerated cutaneous lesions, with no infection signs. This clinical deterioration led to the decision of increasing prednisolone to the pre-admission dosage. Due to a de novo bilateral pleural effusion with dyspnea as the only associated symptom, a CT scan was performed that revealed an incidental finding of a de novo distension of the middle third of the esophagus. Upper endoscopy was then performed, which showed extensive continuous and diffuse esophageal involvement from 20 to 35 cm from the incisors, with severe friability of the mucosa, bullous and necrotic areas, as well as segments with mucosa sloughing, self-limited oozing hemorrhage, and subepithelial hematomas.
AC C
EP
TE D
In this case, the reduction of the prednisolone dosage might have explained the clinical deterioration of the cutaneous lesions, as well as the exuberance of esophageal involvement.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
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EP
TE D
M AN U
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RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
ACCEPTED MANUSCRIPT
Commentary on GIE-D-16-01738R1
SC
RI PT
This case represents an uncommon condition, with a unique GI manifestation. Pemphigoid disorders, which encompass bullous pemphigoid and mucous membrane pemphigoid (MMP), are a spectrum of very rare autoimmune dermatologic conditions. A slight predominance in the elderly and female population has been noted. The pathogenesis involves circulating antibodies that are directed against the basement membrane of squamous epithelium, with subsequent complement activation and inflammatory infiltrate. In the case of bullous pemphigoid, tense bullae on skin surfaces are the consequence of this inflammatory cascade, whereas oral and conjunctival mucosal disease predominates in MMP.
M AN U
In 1978, Eng et al described the first case of extensive esophageal bullae causing upper gastrointestinal hemorrhage. In prior reports of MMP with esophageal involvement, which happens in 4% of cases, patients presented with chest pain, dysphagia, and odynophagia.
AC C
EP
TE D
Interestingly, dissociation between symptoms and endoscopy is evident in this case because the patient was entirely asymptomatic from a GI standpoint. EGD can be challenging in cases like this, due to bullae formation and mucosal sloughing after a light touch of the endoscope of the esophageal mucosa. Gentle movement of the endoscope is recommended in order to prevent adverse events such as bleeding, laceration, and perforation. Therapy is directed toward controlling the underlying immune disease. Systemic steroids are the therapy of choice to achieve remission, as described in this case. However, in cases with brisk GI bleeding causing hemodynamic instability, therapeutic endoscopy can be used to achieve hemostasis. Argon plasma coagulation, which does not require contact with the mucosa, has been successfully used in the past. This case encourages gastroenterologists to be aware of the digestive manifestations of dermatologic diseases and to cautiously perform endoscopy in patients with pemphigoid diseases, even in the absence of overt symptoms. Lady Katherine Mejía Pérez, MD Research Fellow Mayo Clinic Florida Massimo Raimondo, MD Associate Editor for Focal Points