Chronic ulcerative stomatitis

What is it chronic ulcerative stomatitis?

Chronic ulcerative stomatitis was first identified in 1990 as rare. autoimmune disease that presents as painful ulcers in the mouth, often coming and going for many years. Unlike other autoimmune diseases that cause blisters and ulcers in the mouth, it does not respond well to corticosteroids.

Stomatitis means mouth pain.

Who gets chronic ulcerative stomatitis?

Chronic ulcerative stomatitis is a rare disease with less than 100 cases reported so far. It appears to almost exclusively affect white women in late middle age with an average age of onset of 60 years. There are rare reports of chronic ulcerative stomatitis affecting younger men or women. Often, symptoms have been present for many years before the diagnosis is made. It is associated with antibodies against keratinocytes nuclei stratified scaly epithelium. I specify it antigen has been identified as DeltaNp63alpha.

Clinical characteristics of chronic ulcerative stomatitis.

Chronic ulcerative stomatitis presents as painful erosions (shallow ulcers) in the mouth that may look like erosive lichen planus May mimic other autoimmune conditions that cause oral ulcers or erosions, including pemphigus vulgaris and scar pemphigoid, or resembles desquamative gingivitis. Ulcers can occur on the gums, inside the cheeks, or on the tongue. The condition lasts for many years, sometimes coming and going. Very rarely, similar lesions on the genitals or conjunctivals have also been reported. mucous membrane. Skin lesions resembling lichen planus have been reported in some patients. It is still unclear whether chronic ulcerative stomatitis coexists with lichen planus or is a variant of it. The patient is otherwise fine.

How is chronic ulcerative stomatitis diagnosed?

Chronic ulcerative stomatitis is diagnosed by combining clinical and pathological features.

Mucous membrane biopsies of the mouth show nonspecific pathological features, most often resembling lichen planus. However direct immunofluorescence (DIF) shows a speckled pattern of IgG in the lower third of the epithelium (basal and parabasal cell layers).

Indirect immunofluorescence (IIF) in a blood sample detects an IgG and/or IgA autoantibody against keratinocyte nuclei in skin and guinea pig esophageal mucosa but not in HEp2 cells or kidney. This specific antibody It is called stratified epithelium-specific (SES) antinuclear antibody (ANA). The antigen recognized by these autoantibodies It's a 70kDa epithelial nuclear protein and has been identified as DeltaNp63alpha. An ELISA assay has recently been developed to detect specific autoantibodies. The SES-ANA titer is usually high and persists despite the clinical symptoms. remission with treatment Although the titer is often lower with treatment, it does not disappear completely. The titer does not appear to correlate with disease activity. However, the combination of IgG and IgA autoantibodies can indicate serious disease.

The diagnostic criteria have been proposed by Chorzelski et al (1998):

Main criteria

• erosive or exfoliative lesions in the mouth
• characteristic indirect and direct immunofluorescence (IIF and DIF)

Minor criteria

• chronic course with relapses
• woman in the older age group
• response to hydroxychloroquine alone or combined with small doses of cortisone.

Treatment of chronic ulcerative stomatitis

Chronic ulcerative stomatitis usually does not respond to current steroids or oral steroid treatment. However, there is usually an excellent initial response to oral hydroxychloroquine, 200-400 mg/day. This can result in long-lasting remission. However, in the long term, hydroxychloroquine may not prevent relapses and low doses systemic Additionally, corticosteroids and/or dapsone may be required.