Etiology • A pustular eruption usually associated with inflammatory
bowel disease and skin disease • Liver dysfunction (sclerosing cholangitis) may be associated in
some cases.
Clinical Presentation • Mucosal pustules, erythema, edema • Erosions and ulcers may form with serpiginous outlines (“snail
tracks”). • Folds of nodular to hyperplastic tissue (“cobblestoning”)
Microscopic Findings • Neutrophilic and eosinophilic infiltrate into epithelium produc-
bowel disease and skin disease • Liver dysfunction (sclerosing cholangitis) may be associated in
some cases.
Clinical Presentation • Mucosal pustules, erythema, edema • Erosions and ulcers may form with serpiginous outlines (“snail
tracks”). • Folds of nodular to hyperplastic tissue (“cobblestoning”)
Microscopic Findings • Neutrophilic and eosinophilic infiltrate into epithelium produc-
ing microabscesses • Infiltration between epithelial clefts • Epithelial hyperplasia
Diagnosis • Correlation with underlying gastrointestinal disease, such as
the following: • Ulcerative colitis • Crohn’s disease • Sclerosing cholangitis • Malabsorption syndrome
Differential Diagnosis • Oral Crohn’s disease • Pseudomembranous (acute) candidiasis • Melkersson-Rosenthal syndrome • Orofacial granulomatosis • Acanthosis nigricans
Treatment • Successful management of underlying gastrointestinal disease • Local anti-inflammatory agents • Dapsone or sulfapyridine systemically
Prognosis • Correlates with that of systemic disease