Tp. Chorzelski et al., IS CHRONIC ULCERATIVE STOMATITIS AN ENTITY - CLINICAL AND IMMUNOLOGICAL FINDINGS IN 18 CASES, EJD. European journal of dermatology, 8(4), 1998, pp. 261-265
Chronic ulcerative stomatitis (CUS), a rare disease of oral mucosa fou
nd to be associated with unusual antinuclear antibodies reactive exclu
sively with squamous epithelia (squamous epithelium-specific antinucle
ar antibodies - SES-ANA), has been reported to occur almost exclusivel
y in older females and to respond dramatically to antimalarials. We re
port on a large series of 18 cases positive for SES-ANA, 15 of them wi
th active CUS, followed-up for several years. We found that the diseas
e may occur, although rarely, in men and younger females. The antibodi
es persist after clearance of mucosal lesions, are fixed in vivo and a
lso in uninvolved skin and other mucosa, and may occur in patients wit
hout CUS. Thus their pathogenic potential remains to be established. W
e also describe some unusual cases of CUS, one associated with cicatri
cial conjunctivitis, another associated with gluten-sensitive enteropa
thy and bizarre skin lesions, and 3 cases associated with lichen planu
s (LP) or LP-like lesions. The antimalarials, initially highly effecti
ve, even for several months or years, not infrequently proved to be in
sufficient and the relapses responded usually only to combined therapy
with chloroquine and small doses of corticosteroids. SES-ANA antibodi
es are quite specific and present an immunologic marker of this unusua
l autoimmune disease.