[Multiple trichoepitheliomas, XXXX and milia. An entity].

@entity1 @entity4430 @entity2125 @entity4878 @entity26190 @entity5 @entity2347 @entity17142 @entity11885
Four @entity1 from two different families presented with multiple papular @entity17142 of the face associated with @entity11885 and, in one of them, milium. This association, first described by Adamson, has now become classical. It is transmitted as an autosomal dominant trait with variable penetrance. Histochemical studies gave the following results: @entity4430 negative in the two types of @entity5 , phosphorylase weakly positive, @entity2125 diaphorase positive in the basal cells of the @entity17142 and diffusely in @entity2347 . These results suggest that the @entity2347 are of apocrine origin. Using monoclonal antibodies, it has been possible to demonstrate the presence of Langerhans cells in both @entity17142 and @entity2347 . The BL9 and @entity26190 antikeratinocyte monoclonal antibodies were negative, whereas the @entity26190 antibody, which recognizes the 55-57 Kd polypeptides of keratin, marked the suprabasal part of the @entity5 . These results are in favour of incomplete cell differentiation. Treatment with @entity4878 was ineffective, as in all other cases reported. Electrocoagulation or surgical excision om request for cosmetic reasons seem to be only possible treatments.
