Two cases of pyodermatitis-pyostomatitis vegetans.

Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic pustular and vegetating mucocutaneous dermatosis. The oral lesions present as multiple, friable and yellowish pustules, which form "snail tracts" and rupture easily. The cutaneous lesions begin as crusted erythematous papulopustules that coalesce to form large vegetating plaques, usually in the axillae, genital area and scalp. Cutaneous lesions usually develop at the same time as the oral lesions or thereafter. PPV is usually considered as a specific marker for inflammatory bowel disease since the concurrence of PPV and inflammatory bowel disease has been reported in approximately 70% of cases. We report two patients who showed typical clinical and histopathological features of PPV, but were not accompanied by inflammatory bowel disease.