Intercellular and circulating antibodies in patients with dyskeratosis follicularis, Darier's disease

Skin biopsies from 6 patients, and biopsies of the palatal mucosa of 4 of these patients with dyskeratosis follicularis (DF) (Dariers disease) were examined for in vivo bound antibodies by means of a direct immunofluorescence (IF) technique. Antibodies located in the intercellular substance of the epidermis were found in the skin lesions of all patients. Immunoglobulins of the classes IgG, IgA and IgM as well as C3 were found in all lesions. No antibodies reacting with the palatal mucosa were found. Sera from 6 patients with DF and 10 control persons were tested by an indirect IF technique for circulating antibodies. Guinea pig lip and normal oral mucosa and skin were used as antigens. All patients sera and one control serum reacted with the basal cell of the guinea pig lip. Three DF sera--but no control sera--reacted with the basal cells of human oral mucosa. None of the sera reacted with human skin.