A case of pyoderma-pyostomatitis vegetans

Pyoderma-pyostomatitis vegetans (PD-PSV) is a rare, benign, eosinophilic pustular and vegetating mucocutaneous disease characterized by skin lesions which typically involve the axillary and genital regions, the face and the scalp. PD-PSV was at first regarded as a subtype of bullous disease. However, due to the lack of abnormality under a immunofluorescent microscope, it could be diffentiated from bullous disease. A 48-year woman presented with a 6-month history of sharply-outlined, exudative, papillomatous and vesiculopustular vegetating plaques on the perioral, umbilicus and nasal mucosa, tips of her fingers and toes and perianal region. A skin biopsy taken from the lip and umbilicus showed papillary dermal edema and focal inflammatory cell infiltration composed of many eosinophils, intraepithelial microabscesses, focal spongiosis, and exocytosis. No abnormalities were found during an immunofluorescence study. The lesions were almost cleared with 20 mg of triamcinolone and 200 mg of cyclosporin medication during a 3-month treatment period.