Chronic ulcerative stomatitis (CUS) has recently been described as a n
ew disease entity characterized by chronic ulceration of oral mucosa w
hich responds to treatment with hydroxychloroquine. It has a particula
r type of stratified epithelium-specific, antinuclear autoantibody as
an immunological marker. Twelve cases have been reported in the litera
ture. We present a 40-year-old woman with an Ii-year history of chroni
c oral ulcerations. Other dermatological diseases, including oral lich
en planus, pemphigus vulgaris and cicatricial pemphigoid, as well as b
ullous lupus erythematosus, were excluded. The clinical diagnosis of C
US was confirmed on the grounds of the immunological and ultrastructur
al findings, The lesions initially responded to high doses of systemic
corticosteroids but relapsed promptly after dose reduction. Dapsone w
as ineffective. Hydroxychloroquine, given at a dosage of 200-400 mg/da
y, led to a complete and long-lasting remission.