Chronic ulcerative stomatitis with stratified epithelium-specific antinuclear antibodies

Background. In 1990 a new disease-associated antinuclear antibody was first recognized as a specific immunologic marker for a chronic form of ulcerative stomatitis (CUS). Methods. Another case is reported herein and the subject of chronic ulcerative stomatitis with stratified epithelium-specific antinuclear antibodies (SES-ANA) is reviewed, Intraoral biopsies from this patient were submitted for microscopic examination and direct immunofluorescence. Indirect immunofluorescence studies were also performed. Serial SES-ANA liters were obtained with the patient on maintenance treatment with hydroxychloroquine. A skin biopsy of a recent lichenoid eruption was obtained and skin explants grown in the serum of this patient were studied in tissue culture with reference to SES-ANA binding and complement fixation. Results. Biopsy and serum studies confirmed a diagnosis of CUS with SES-ANA in the patient reported. Skin biopsy showed lichen planus. The patient was treated with hydroxychloroquine with a favorable response. Serial SES-ANA titers did not parallel the disease activity. Among the substantive observations made from skin explants cultured in the serum of this patient was widespread fixation of C3 to the nuclei of basal cells. Conclusions. The case described herein extends the findings in CUS to include lichenoid skin lesions. Records show that at least four of 11 cases of CUS had skin lesions, whereas all had oral lesions. Stratified epithelium-specific antinuclear antibodies serve as the key marker of CUS. Skin explants grown in the serum of this CUS patient bind SES-ANA in tissue culture. Sections of explants fix complement. Titers of SES-ANA ANA have been reported to parallel disease activity in one case, but not in the present case. Thus, there appears to be case-to-case variation. The treatment of choice for CUS is hydroxychloroquine.