Pyodermatitis-pyostomatitis vegetans is a rare inflammatory dermatosis. There is a strong association between pyodermatitis-pyostomatitis vegetans and inflammatory bowel disease, particularly ulcerative colitis. Herein, we report a case of pyodermatitis-pyostomatitis vegetans with positive direct immunofluorescence staining findings and review the literature for the past 18 years to characterize the disease, its epidemiologic characteristics, its associations, and the pathology and direct and indirect immunofluorescence findings. The total number of cases was 38, including 22 men and 16 women, with an average age of forty. Direct immunofluorescence staining had been performed for 32 patients, of which 12 had positive findings. Of those with positive direct immunofluorescence, 6 patients showed IgA cell surface staining. A recent approach suggests that these immunological findings may not be accidental and indicates a possible overlap with autoimmune bullous diseases discussed in this review.