Pyodermatitis-pyostomatitis vegetans: a clinical course of two decades with response to cyclosporine and low-dose prednisolone

Pyodermatitis-pyostomatitis vegetans is a rare, polymorphous inflammatory disorder of the skin and oral mucosa first described by Hallopeau in 1898. On the skin papules, pustules and reddish brown annular vegetating plaques develop, most frequently in the intertriginous areas. In the mouth, yellowish flat ulcerations arise, typically in the shape of "snail tracks". The association with inflammatory bowel disease is very common. An unusual case with a chronic relapsing course of 2 decades is presented. Gastrointestinal inflammation was absent. Prednisolone in high and medium doses suppressed most lesions. Various attempts with other drugs (dapsone, isotretinoin, azathioprine) to reduce the corticosteroid dose failed. This is the first report of the successful treatment of pyodermatitis-pyostomatitis vegetans with cyclosporin A, which proved to be highly effective in this regard. The unknown aetiopathology of pyodermatitis-pyostomatitis vegetans is discussed.