ORAL MANIFESTATIONS OF DARIERS-DISEASE - A CLINICAL AND PATHOLOGICAL-STUDY

Patients with Darier's disease may present oral lesions which occur in about 50% of affected patients. The palate, gingiva, buccal mucosa an d the tongue are most commonly affected. This study reports the clinic al and pathological features of the oral manifestations of Darier's di sease. The pathology of early reddish spots reveals ortho-parakeratoti c hyperkeratosis with focal hypergranulosis and acanthosis. The pathol ogy of established white papules shows the typical features of acantho lytic dyskeratosis with suprabasal clefts containing shows the acantho lytic dyskeratotic keratinocytes. Our study indicates that although ac antholytic dyskeratosis is a pathological marker of established oral l esions of Darier's disease may not be detectable in early oral spots. The absence of acantholytic dyskeratosis in early lesions is not exclu sive to mouth pathology, it has been described in the early nail lesio ns of patients with Darier's disease. These findings of acantholytic d yskeratosis should not be considered an absolute criterion for excludi ng a possible diagnosis of Darier's disease.