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Oral Diseases / Volume 25, Issue 6

REVIEW ARTICLE

Chronic ulcerative stomatitis: A

comprehensive review and proposal for
diagnostic criteria

Lorenzo Azzi ! , Michele Cerati , Maurizio Lombardo

… See all authors ""

First published: 20 November 2018

https://doi.org/10.1111/odi.13001

Abstract
Chronic ulcerative stomatitis (CUS) is an
immune‐mediated disorder characterized
by oral erosions and ulcers usually
refractory to conventional treatments. The
disease often involves middle‐aged and
older women with painful lesions
sometimes resembling those of erosive
oral lichen planus (OLP). The most a!ected
sites are the buccal mucosa, the gingiva
and the tongue, but the skin is involved in
22.5% of cases. Histopathologic features in
CUS are non‐speci"c and indistinguishable
from those of OLP, with the exception of
the presence of a mixed in"ltrate
composed of lymphocytes and plasma
cells. Direct immuno#uorescence (DIF)
analysis reveals the presence of strati"ed
epithelium‐speci"c antinuclear antibodies
(SES‐ANA) in the lower third of the
epithelium. The IgG antibodies detected on
DIF are directed against the ∆Np63α
isoform of p63 expressed in the nuclei of
the epithelial basal cells. A distinguishing
feature of CUS is the low response to
conventional corticosteroid therapy and
the good outcome with
hydroxychloroquine at the dosage of
200 mg/day or higher dosages. This paper
presents a comprehensive review of CUS
and is accompanied by a new case report
(the 73rd case) and a proposal for updated
diagnostic criteria.

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