What is chronic ulcerative stomatitis?
Chronic ulcerative stomatitis was first identified in 1990 as a rare autoimmune disease that presents as painful ulcers in the mouth, often coming and going for many years. Unlike other autoimmune diseases that cause mouth blisters and ulcers, it does not respond well to corticosteroids.
Stomatitis means a sore mouth.
Who gets chronic ulcerative stomatitis?
Chronic ulcerative stomatitis is a rare disease with less than 100 cases so far reported. It seems to almost exclusively affect white women in late middle age with an average age at onset of 60 years. There are rare reports of chronic ulcerative stomatitis affecting males or younger women. Often the symptoms have been present many years before the diagnosis is made. It is associated with antibodies against keratinocyte nuclei of stratified squamous epithelium. The specific antigen has been identified as DeltaNp63alpha.
Clinical features of chronic ulcerative stomatitis
Chronic ulcerative stomatitis presents as painful erosions (superficial ulcers) in the mouth which can resemble erosive lichen planus. It may mimic other autoimmune conditions that cause mouth ulcers or erosions including pemphigus vulgaris and cicatricial pemphigoid, or resemble a desquamative gingivitis. The ulcers can occur on the gums, inside the cheeks or on the tongue. The condition lasts many years, sometimes coming and going. Very rarely similar lesions have also been reported on the genital or conjunctival mucosa. Skin lesions resembling lichen planus have been reported in some patients. It is not yet clear if chronic ulcerative stomatitis coexists with lichen planus or is a variant of it. The patient is otherwise well.
How is chronic ulcerative stomatitis diagnosed?
Chronic ulcerative stomatitis is diagnosed on the combination of clinical and pathological features.
Mucosal biopsies from the mouth show nonspecific pathological features, most often resembling lichen planus. However direct immunofluorescence (DIF) shows a speckled pattern of IgG in the lower one third of the epithelium (basal and parabasal cell layers).
Indirect immunofluorescence (IIF) on a blood sample detects an IgG and/or IgA autoantibody against keratinocyte nuclei in skin and guinea pig oesophageal mucosa but not in HEp2 cells or kidney. This specific antibody is called stratified epithelium-specific (SES)-antinuclear antibody (ANA). The antigen recognised by these autoantibodies is a 70kDa epithelial nuclear protein and has been identified as DeltaNp63alpha. An ELISA test assay has recently been developed to detect the specific autoantibodies. The SES-ANA titre is usually high and persists despite clinical remission with treatment. Although the titre is often lower with treatment it does not disappear completely. The titre does not appear to correlate with disease activity. However the combination of both IgG and IgA autoantibodies may indicate severe disease.
Diagnostic criteria have been proposed by Chorzelski et al (1998):
- erosive or exfoliative lesions in the mouth
- characteristic indirect and direct immunofluorescence (IIF and DIF)
- chronic course with relapses
- female in the older age group
- response to hydroxychloroquine alone or combined with small doses of cortisone.
Treatment of chronic ulcerative stomatitis
Chronic ulcerative stomatitis typically does not respond to topical steroids or oral steroid treatment. However there is usually an excellent initial response to oral hydroxychloroquine, 200-400mg/day. This may result in a longlasting remission. However in the longterm, hydroxychloroquine may not prevent relapses and low dose systemic corticosteroids &/or dapsone may be required in addition.