A case of pemphigus vulgaris associated with ulcerative colitis

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.

Persistent Scattered Pustules: Vesiculopustular IgA Pemphigus Arising in a Patient With Ulcerative Colitis

“…IgA pemphigus is uncommon and typically presents with fewer lesions than pemphigus vulgaris. [1][2][3] IgA pemphigus is also known as intraepidermal neutrophilic IgA dermatosis, intercellular IgA vesiculopustular dermatosis, or intraepidermal IgA pustulosis. Direct immunofluorescence reveals intercellular IgA deposition in 97.0% of patients.…”

Cell-Matrix Interactions Contribute to Barrier Function in Human Colon Organoids

“…Finally, there is another group of diseasesepidermolysis bullosa and related conditions-that are manifestations of mutations in various basement membrane, desmosomal/hemidesmosomal and keratin genes (61). At the same time, there are case reports and studies that provide evidence of an association between bullous pemphigoid and inflammatory bowel disease (62)(63)(64). At this point, we can only speculate as to whether optimizing the expression of multiple cell-cell and cell-matrix adhesion molecules in an individual might overcome, at least in part, the consequences of a functionmodifying mutation in one or another critical component.…”

Cell-matrix adhesion contributes to permeability control in human colon organoids

“…Finally, there is another group of diseases -epidermolysis bullosa and related conditions -that are manifestations of mutations in various basement membrane, desmosomal / hemidesmosomal and keratin genes (50). At the same time, there are case reports and studies that provide evidence of an association between bullous pemphigoid and inflammatory bowel disease (51)(52)(53). At this point, we can only speculate as to whether optimizing the expression of multiple cell-cell and cell-matrix adhesion molecules in an individual might overcome, at least in part, the consequences of a function-modifying mutation in one or another critical component.…”