Ear involvement in ligneous conjunctivitis: a rarity or an under-diagnosed condition?

Conjunctivitis lignosa, a rare affliction of the conjunctiva, is sometimes associated with other disturbances. We present two children with concurrent conjunctivitis lignosa and ear involvement. In these two cases, there were histopathologically verified ligneous changes of the middle ears. Routine haematoxylin and eosin, van Gieson, periodic acid-Schiff (PAS) and alcian blue staining of specimens from the eyes and middle ears revealed findings typical for ligneous conjunctivitis. In addition, new histochemical and immunohistochemical studies for glycosaminoglycans on specimens from the eyes and middle ears showed that the accumulations of the amorphous, cell-deficient material stained strongly but heterogeneously for hyaluronic acid and weakly but uniformly for keratin sulphate. The staining for other glycosaminoglycans, e.g. chondroitin-4-sulphate and dermatan sulphate was confined to vessels and areas rich in collagen fibres and fibroblasts. In patients with conjunctivitis lignosa, the ear involvement may remain undiagnosed due to its resemblance to secretory otitis media with effusion. Since isolated ear involvement may occur, we advocate biopsies for routine haematoxylin and eosin, and specific staining for hyaluronic acid and keratin sulphate, also in children with protracted, refractory otitis media with atypical effusion.