Colonic involvement in pemphigus vulgaris: a rare cause of chronic diarrhea

Table of Contents

Pemphigus vulgaris is an autoimmune disorder characterized by epithelial blistering affecting mostly the skin and oral mucosa. The gastrointestinal tract is rarely involved; when it is, localization is usually to the esophagus or rectum [1] [2]. Colonic involvement is exceedingly rare, having been anecdotally described as the cause of hematochezia [3] or associated with ulcerative colitis [4]. Bullous colon lesions in a patient with bullous pemphigoid have also been described [5].

A 62-year-old white man was referred to our unit by the consultant nephrologists because of persistent diarrhea and hematochezia. The patient’s past medical history included arterial hypertension, mild chronic obstructive pulmonary disease, and “minimal lesion nephropathy.” Colonoscopy showed multiple bullous lesions throughout the colon ([Figure 1]). Histologic analysis documented dissociation between epithelium and stroma with eosinophilic and lymphocellular infiltration of the lamina propria, around the basement membrane zone ([Figure 2]). The diagnosis of pemphigus was confirmed with indirect immunofluorescence. Treatment with prednisone (1 mg/kg per day) and azathioprine (100 mg per day) was instituted with rapid clinical improvement. Complete remission of the symptoms was achieved in 6 weeks and the patient’s condition since has been stable over a follow-up of almost 5 years. Immunofluorescence studied were repeated periodically and gave negative results

Figure 1 a, b Endoscopic appearance with multiple flattened, whitish bullous lesions disseminated throughout the colon.

Figure 2 Histologic study revealed a suprabasilar acantholysis, eosinophilic infiltrate into the lamina propria and subepithelial bullae (H&E, × 20) with b dissociation of the epithelium from the stroma and sparse eosinophilic and lymphomonocytic infiltrate of the lamina propria (H&E, × 400).

In the present case, gastrointestinal symptoms were the prevailing clinical picture, with lesions of the intestinal mucosa occurring long before those of the oral mucous membranes. The endoscopic pattern of scattered erythema, whitish flattened plaques, and flaccid bullae, and the clinical picture of persistent diarrhea, with or without rectal bleeding, should raise a suspicion of colonic involvement in pemphigus. Biopsies typically show acantholysis with inflammation of the submucosal tissues. The diagnosis is confirmed by means of indirect immunofluorescence studies and detection of IgG autoantibodies to desmoglein 3 by enzyme-linked immunosorbent assay.

Treatment is based on high-dose corticosteroids. Adjunctive therapy with immunosuppressive agents or intravenous immunoglobulins has proved successful and safe in steroid-resistant forms.

Endoscopy_UCTN_Code_CCL_1AD_2AD

References

• 1 Gomi H, Akiyama M, Yakabi K, Nakamura T, Matsuo I. Oesophageal involvement in pemphigus vulgaris.  Lancet. 1999;  354 1794
• 2 Stone D D. Rectal lesions and toxic dilatation of the colon in a case of pemphigus vulgaris.  Am J Dig Dis. 1977;  16 163-166
• 3 Schwermann M, Lechner W, Elsner C, Kirchner T. Pemphigus vulgaris with involvement of duodenum and colon.  Z Hautkr. 1988;  63 101-104
• 4 Kacar S, Sezgin O, Sahin T. Pemphigus vulgaris and ulcerative colitis [letter].  Am J Gastroenterol. 2002;  97 507-508
• 5 Sachsenberg-Studer E M, Runne U, Wehrmann T. et al . Bullous colon lesions in a patient with bullous pemphigoid.  Gastrointest Endosc. 2001;  54 104-108