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Chronic Ulcerative Stomatitis
What is Chronic Ulcerative Stomatitis?

Chronic Ulcerative Stomatitis (CUS) was first identified in
1990 as a rare
auto-immune condition that presents as
painful ulcers in the mouth, often coming and going for
many years.  Unlike other
auto-immune diseases that
cause mouth blisters and ulcers, it does not respond well
to
corticosteroids.

(
Stomatitis means a sore mouth).


Who gets Chronic Ulcerative Stomatitis?

CUS is rare 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 CUS 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.


Clinical Features of Chronic Ulcerative Stomatitis

CUS presents as painful erosions (superficial ulcers) in the
mouth which can resemble
erosive lichen planus.  It may
mimic other
auto-immune 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
oral lichen planus have been
reported in some patients.  It is not yet clear if CUS
co-exists with
oral lichen planus or is a variant of it.

The patient is otherwise well.
Last Updated 1st August 2012
Photo of Chronic Ulcerative Stomatitis affecting the lower lip
How is Chronic Ulcerative Stomatitis diagnosed?

CUS is diagnosed on the combination of clinical and pathological features.

Mucosal biopsies from the mouth show non-specific pathology features, most often
resembling
oral lichen planus.  However, direct immuno-fluorescence (DIF) shows
a speckled pattern of IgG (
immunoglobulin G, a type of antibody) in the lower one
third of the
epithelium (basal & para-basal cell layers).

Indirect immuno-fluorescence (IIF) on a blood sample detects an IgG and / or IgA
(another type of immunoglobulin / antibody) auto-antibody against keratinocyte
nuclei in skin.  This specific antibody is called
Stratified Epithelium-Specific (SES)-
Anti-Nuclear Antibody (ANA).  The antigen recognised by these auto-antibodies is
a 70 kilo Dalton
epithelial nuclear protein and has been identified as Delta Np63
alpha
(ΔNp63α).

An
ELISA test assay has recently been developed to detect the specific auto-
antibodies.  The
SES-ANA (Stratified Epithelium Specific-Anti-Nuclear Antibody)
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 auto-antibodies may indicate severe disease.


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

Major Criteria

•        erosive or exfoliative lesions in the mouth
•        characteristic
indirect and direct immuno-fluorescence (I-IF & D-IF)


Minor Criteria

•        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

CUS 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 long-lasting remission.  However in the
long term, hydroxychloroquine may not prevent relapses and low dose systemic
corticosteroids & / or dapsone may be required in addition.



Websites:

New Zealand Dermatological Society (DermNet NZ)

Br J Dermatol

J Dental Research