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 d isorder of the skin and oral mucosa first described by Hallopeau in 1898, O n the skin papules, pustules and reddish brown annular vegetating plaques d evelop, most frequently in the intertriginous areas. Tn the mouth, yellowis h flat ulcerations arise, typically in the shape of "snail tracks". The ass ociation with inflammatory bowel disease is very common. An unusual case wi th a chronic relapsing course of 2 decades is presented. Gastrointestinal i nflammation was absent. Prednisolone in high and medium doses suppressed mo st lesions, Various attempts with other drugs (dapsone, isotretinoin, azath ioprine) to reduce the corticosteroid dose failed. This is the first report of the successful treatment of pyodermatitis-pyostomatitis vegetans with c yclosporin A, which proved to be highly effective in this regard. The unkno wn aetiopathology of pyodermatitis-pyostomatitis vegetans is discussed.