Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Original Article
doi: 10.4103/0378-6323.29337
PMID: 17179616

Study of upper gastrointestinal tract involvement in pemphigus by esophago-gastro-duodenoscopy

P Narasimha Rao1 , Aruna Samarth1 , Sanjeev J Aurangabadkar1 , Bajrang Pratap2 , TSS Lakshmi1
1 Department of Dermatology, Osmania Medical College, Hyderabad, India
2 Department of Gastroenterology, Osmania Medical College, Hyderabad, India

Correspondence Address:
P Narasimha Rao
B-48, Income Tax Colony, Mehdipatnam, Hyderabad - 500 028
How to cite this article:
Rao P N, Samarth A, Aurangabadkar SJ, Pratap B, Lakshmi T. Study of upper gastrointestinal tract involvement in pemphigus by esophago-gastro-duodenoscopy. Indian J Dermatol Venereol Leprol 2006;72:421-424
Copyright: (C)2006 Indian Journal of Dermatology, Venereology, and Leprology


Introduction: Involvement of upper gastrointestinal tract in pemphigus vulgaris is not uncommon. Aim: To study the involvement of upper gastrointestinal tract (UGIT) with the help of esophago-gastro-duodenoscopy (EGD) in patients of vesiculobullous dermatoses with emphasis on pemphigus vulgaris. Methods: Forty-two patients (M-22, F-20) with vesiculobullous dermatoses, diagnosed on the basis of clinical features and skin histopathology as pemphigus vulgaris (PV)-40 patients and pemphigus foliacieus (PF)-2 patients were included in the study. The EGD was performed and mucosa of the esophagus, stomach and first part of the duodenum were examined. Mucosal biopsies were taken from the lower esophagus in 26 patients of PV and studied after H and E staining. Results: On EGD, esophageal involvement was seen in 67% patients of PV (27/40). Of these, Grade I esophagitis was observed in seven, Grade II in 11, Grade III in four and Grade IV involvement was seen in five patients of PV. Three PV patients had associated esophageal candidiasis. Involvement of esophageal mucosa was also observed in one out of two patients of PF. Gastric mucosa was involved in 52% and duodenal mucosa in 20% of PV patients. Acantholysis was observed in seven out of 26 (27%) esophageal biopsies of PV patients. Two patients of PV vomited a tube-like structure, indicative of 'esophagitis dissecans superficialis'. The involvement of the gastric mucosa in patients with history of oral corticosteroid intake (60%) was compared to the group without history of oral corticosteroids (30%). Conclusion: Among PV patients under study, significant involvement of oral (87%), esophageal (67%), gastric (52%) and duodenal mucosa (20%) was observed.
Keywords: Esophago-gastro-duodenoscopy, Esophagus, Gastric mucosa, Pemphigus vulgaris, Vesiculobullous disorders


Vesiculobullous dermatoses, particularly the pemphigus group of disorders, are important for the morbidity associated with the involvement of skin and mucous membranes. In the diseases where they are involved, mucosal involvement is not limited only to the oral cavity but extends to the esophagus as well. This upper gastrointestinal tract (UGIT) involvement adds to the morbidity and complications of the disease. Esophago-gastro-duodenoscopy (EGD) or upper gastrointestinal endoscopy is one of the most commonly performed endoscopic procedures. In the present study, we examined patients of pemphigus for the involvement of the UGIT with the help of this procedure and in some patients mucosal biopsies from esophagus were studied.


A total of 42 patients (22 males, 20 females) presenting with pemphigus to the Department of Dermatology, Osmania General Hospital, Hyderabad, India, between January 1995 and December 1999 were included in the study. Both untreated and partially treated patients of all age groups were included in the study after fully informed written consent was taken. Detailed clinical examination was performed and recorded. Skin biopsies were taken from active skin lesions in all patients to confirm clinical diagnosis. Direct or indirect immunofluoroscence was not performed due to lack of facilities. All the patients were subjected to EGD at the Department of Gastroenterology, Osmania General Hospital, Hyderabad. The severity of involvement of the esophageal mucosa was graded on a scale of I to IV based on the endoscopic findings [Table - 1]. Similarly, the macroscopic findings under EGD of the gastric and duodenal mucosa were recorded. Biopsies from the esophageal mucosa were taken from active lesions involving the lower esophagus in 26 patients.


Out of the 42 patients, 22 were male and 20, female. The age distribution of the patients was as follows: five patients were in the 10-20 years age group, 23 were in the 20-45 years age group and 14 were aged over 45 years.

The clinical diagnosis was PV in 40 patients and PF in two patients. The duration of the disease in these patients ranged from one month to four years.

On clinical examination, the oral mucosa was involved in 35 out of 40 patients of PV patients and in one out of two patients of PF. It was predominantly in the form of erosions and ulcers of the buccal mucous membrane; in 12 patients, the lips were also involved. The majority of the patients with oral ulcers complained of burning pain, dysphagia and odynophagia. One patient of PV gave a history of hematemesis.

The results of EGD are given in [Table - 2]. On EGD, esophageal involvemnt was observed in 27 out of 40 PV patients and one out of 2 PF patients. Three patients with Grade IV esophagitis of these patients also had associated esophageal candidiasis. On EGD, gastric mucosal involvement was observed in 21 patients and duodenal mucosal involvement in eight [Table - 2]. The types of lesions involving the antrum and body of the stomach were ulcers, erosions and severe diffuse gastritis. In the duodenum, duodenitis, small ulcers and deformed cap were the types of involvement. None of the PF patients showed gastric or duodenal involvement. Esophageal mucosal biopsies were taken in 26 patients with esophageal involvement [Table - 3]. Twenty eight of the 42 patients had taken oral corticosteroids for up to six months for their disease before presenting to us and undergoing EGD. Gastric mucosal involvement was commoner in patients taking oral corticosteroids than in those who had not, while esophageal involvement was equally common in both these groups. Another significant observation was the vomiting of a tube-like structure, indicative of esophagitis dissecans superficialis, by two patients of PV.


An assessment of UGIT involvement in pemphigus may help determine the extent and severity of the disease and may guide the approach to therapy.[1] We observed esophageal involvement in 27 out of 40 patients of PV (67%). In a study from India, 18 out of 25 (72%) PV patients had esophageal involvement.[2]

All PV sera have anti-Dsg3 antibodies and over 50% have anti-Dsg1 antibodies. Antibodies against both Dsg3 and Dsg1 are required to produce significant blistering in the skin.[3] Pemphigus vulgaris entirely limited to the mucous membranes is seen in those with no reactivity to Dsg1.[4] In a majority of the patients, painful oral mucous membrane erosions are the presenting sign of PV.[5] In our study, 87% of patients of PV presented with oral lesions.

About half of the patients of PV have esophageal involvement but this is usually asymptomatic.[1] The whole or segments of the esophageal mucosa can be involved.[6] Patients may complain of odynophagia, dysphagia and occasionally gastrointestinal bleeding. In our study, 36 patients with oral mucosal ulcers and erosions (including 27 with esophageal involvement) presented with varying grades of dysphagia and odynophagia. One patient also gave a history of hematemesis. Esophageal involvement may become evident in the absence of cutaneous manifestations when the latter are in remission or during treatment.[7],[8]

Very rarely, bullous diseases may affect the esophagus in such a manner that there is sloughing of the entire mucous membrane, leading to the formation of a mucosal cast (′esophagitis dissecans superficialis′), which may be vomited.[9] Such a condition was observed in two of our patients; one of them who vomited this cast did so on the second day after his EGD, which probably precipitated the cast′s dislodgement.

Acantholysis was observed only in seven out of 26 esophageal biopsies (27%) in our study. In similar studies, acantholysis was observed in 36% and 50% of PV patients.[1],[10] We could not perform direct immunofluorescence (DIF) on esophageal biopsies although studies have shown that DIF is positive in all PV patients biopsied from any part of the esophageal mucosa, demonstrating that immune deposition occurs along the entire length of the esophagus irrespective of the degree of clinical involvement.[10]

A finding either to unreported was the involvement of the gastric (52%) and duodenal (20%) mucosa on EGD in our patients of PV. Stress ulcerations of gasric mucosa are known to occur in very ill patients due to the loss of barrier protection during critical illness. We did not perform any histological or immunological study of gastric or duodenal mucosal lesions. We could only presume that these lesions if not due to the disease, could be due to the effects of oral corticosteroids as many patients gave history of their intake. It has been observed that lack of correlation between the patients′ subjective complaints and endoscopic findings indicates the importance of endoscopy in assessing gastroduodenal lesions in patients on corticosteroid therapy.[11] In our study, 28 out of 42 patients gave a history of taking oral steroids for varying periods before undergoing EGD. Gastric mucosal involvement was higher in this group of patients (60%) than in those who had not taken oral steroids (28%), indicating that steroid intake played a role.

In patients with severe PV, stratified squamous mucosal surfaces such as laryngeal, esophageal, conjunctival, urethral, vaginal, cervical and anal mucosa are known to be involved.[12] However, there is a recent report[13] of involvement of other non-stratified mucosal surfaces of the intestinal tract.

Three patients in the present study demonstrated candidiasis of esophagus on EGD. Prolonged use of corticosteroids, including topical preparations, have reportedly led to oropharyngeal and esophageal candidiasis in otherwise healthy individuals.[14] Hypochlorhydria due to acid-suppressive medications, which are usually given as adjuvants along with corticosteroids, predisposes to candidial esophagitis by removing the cleansing effect of normal periodic acid reflux events.[15]

In the present study, we have observed a high proportion of oral and esophageal mucosal involvement in PV patients, apart from involvement of the stomach and duodenum. Study of the esophagus by EGD in PV and other vesiculobullous disorders is not only useful in assessing the extent of the involvement of UGIT but also in evaluating the efficacy of the treatment instituted. The lesions in the stomach and duodenum observed in patients of PV in our study cannot be attributed to the disease as the histopathology or immunopathology of these lesions was not performed. However, reports of the involvement of the colon in a patient of pemphigus[13] entails that further studies are required to determine the extent and nature of involvement of gastro intestinal mucosa in these disorders.

Eliakim R, Goldin E, Livshin R, Okon E. Esophageal involvement in pemphigus vulgaris. Am J Gastroenterol 1988;83:155-7.
[Google Scholar]
Sawant P, Satarkar RP, Chopda N, Amladi ST, Nanivadekar SA, Deodhar KP. Esophageal involvement in pemphigus vulgaris. Indian J Gastroenterol 1994;13:133-4.
[Google Scholar]
Miyagava S, Amagei M, Iida T, Yamamoto Y, Nishikawa T, Shirai T. Late development of antidesmoglein-1 antibodies in pemphigus vulgaris: Correlation with disease progression. Br J Dermatol 1999;141:1084-7.
[Google Scholar]
Ding X, Aoki V, Mascaro JM, Lopez-Swiderski A, Diaz LA, Fairley JA. Mucosal and muco cutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. J Invest Dermatol 1997;109:592-6.
[Google Scholar]
Stanley JR. Pemphigus. In : Freedberg IM, Eisen AZ, Wolff K, Austin KF, Goldsmith LA, Katz SI, et al . editors. Fitzparticks's Dermatology in General medicine, 5th ed. New York: Mcgrew-Hill; 1999. p. 654-66.
[Google Scholar]
Mignogna MD, Lo Muzio L, Galloro G, Satriano RA, Ruocco V, Bucci E. Oral pemphigus: Clinical significance of esophageal involvement: Report of eight cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:179-84.
[Google Scholar]
Goldin E, Lijovetzky G. Esophageal involvement by pemphigus vulgaris. Am J Gastroentrol 1985;80:828-30.
[Google Scholar]
Mobacken H, Al Karawi M, Mohamed A, Coode P. Oesophageal Pemphigus vulgaris. Dermatologica 1988;176:266-9.
[Google Scholar]
Schissel DJ, David-Bajar K. Esophagitis dissecans superficialis associated with pemphigus vulgaris. Cutis 1999;63:157-60.
[Google Scholar]
Trattner A, Lurie R, Leiser A, David M, Hazaz B, Kadish U, et al . Esophageal involvement in pemphigus vulgaris: A clinical, histologic and immunopathologic study. J Am Acad Dermatol 1991;24:223-6.
[Google Scholar]
Okada M, Fuchigami T, Iida M, Omae T, Akagi K, Onoyama K. Adreno-corticosteroid therapy and gastroduodenal lesions. Gastrointest Endosc 1985;31:188-90.
[Google Scholar]
Pye RJ. Bullous eruptions. In : Champion RH, Burton JL, Ebling FJ, editors. Rook's Text Book of Dermatology. 5th ed. New York: Blackwell Publications; 1992. p. 1638-48.
[Google Scholar]
Bruckner AL, Fitzpatrick JE, Hashimoto T, Weston WL, Morelli JG. Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: A novel variant of IgA pemphigus? Pediatr Dermatol. 2005;22:321-327
[Google Scholar]
Baehr PH, McDonald GB. Esophageal disorders caused by infection, systemic illness, medication, radiation and trauma. In : Feldman M, Scharschmidit BR, Sleisenger MH, editors. Sleisenger and Fordtran's gastrointestinal and liver diseases, 6th ed. London: WB Saunders; 1998. p. 519-39.
[Google Scholar]
Larner AJ, Lendrum R. Oesophageal candidiasis after omeprazole therapy. Gut 1992;33:860-1.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections