Intest Res.  2018 Jan;16(1):147-150. 10.5217/ir.2018.16.1.147.

A case of pemphigus vulgaris associated with ulcerative colitis

• Seo JW
• Park J
• Lee J
• Kim MY
• Choi HJ
• Jeong HJ
• Lee JW
• Jung SY
• Kim WK

• KMID: 2402659
• DOI: http://doi.org/10.5217/ir.2018.16.1.147

Abstract

Pemphigus vulgaris is an autoimmune bullous disorder characterized by the production of autoantibodies against the intercellular space of the epithelium. It has rarely been reported in association with inflammatory bowel disease. Ulcerative colitis is one of the forms of inflammatory bowel disease. A 62-year-old woman who had been treated for ulcerative colitis for 16 years developed pruritic bullae on the skin of her face and body. Histological findings and direct immunofluorescence examination of the skin showed pemphigus vulgaris. She was treated with systemic steroids, mesalazine, and azathioprine. Her cutaneous lesions have remained in remission and her ulcerative colitis has remained well-controlled. The relationship between pemphigus vulgaris and ulcerative colitis is unclear. An autoimmune response has been suspected in the pathogenesis of ulcerative colitis. Pemphigus vulgaris is also associated with an autoimmune mechanism. To our knowledge, this is the first case of ulcerative colitis associated with pemphigus vulgaris reported in Korea. The association may be causal.

Keyword

Colitis, ulcerative; Pemphigus; Autoimmunity

MeSH Terms

Autoantibodies
Autoimmunity
Azathioprine
Colitis, Ulcerative*
Epithelium
Extracellular Space
Female
Fluorescent Antibody Technique, Direct
Humans
Inflammatory Bowel Diseases
Korea
Mesalamine
Middle Aged
Pemphigus*
Skin
Steroids
Ulcer*
Autoantibodies
Azathioprine
Mesalamine
Steroids

Figure

• 

  Fig. 1 Cutaneous features of the patient. (A) Crust formation on nose was appeared after scratching. (B) Pruritic erythematous bullae filled with serous exudate showed on the skin of lower leg.

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  Fig. 2 Pathologic finding of the erythematous bullae of lower leg. Microscopic findings showed a suprabasilar cleft and vesicle (white arrow) with 1 to 2 layers of suprabasal keratinocytes attached to basement membrane forming part of floor of the cleft. Dermal papillae were prominent with acantholytic basal cells (black arrow) (H&E, ×400).

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  Fig. 3 Colonoscopic findings. (A) Colonoscopy showed diffuse erythema, edematous mucosa with multiple ulceration in descending colon. (B) Follow-up colonoscopy showed focal erythema and loss of vascularity.

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