Stephen K. Young

Familial gigantiform cementoma: Classification and presentation of a large pedigree

Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. Confusion exists about the relationship of gigantiform cementoma to florid osseous dysplasia, cementifying fibroma, and diffuse chronic sclerosing osteomyelitis. It has been unclear whether gigantiform cementoma should be accorded the status of a separate entity. In this article, we report our findings on a family that, over five generations, has exhibited clinical, radiographic, and/or histologic findings consistent with the designation familial gigantiform cementoma. This pedigree consists of 55 members. Significant heterogeneity in expression of this trait was noted. The pattern of occurrence of the trait is consistent with an autosomal dominant mode of inheritance with variable expressivity of the phenotype. We suggest that familial gigantiform cementoma should be recognized as a separate entity.

Desmoplastic fibroma of the mandible in a child: report of a case

A 6-year-old girl was seen with a history of progressive trismus and expansion of the right mandible over a 9-month period. Desmoplastic fibroma was diagnosed on biopsy. This report reviews the diagnostic criteria and surgical management of this uncommon primary bone tumor of the maxillofacial region.

Generalized eruptive keratoacanthoma

Two forms of keratoacanthoma are presently recognized: a solitary form and a multiple form. The multiple form has two variants: multiple self-healing epitheliomas of skin or Ferguson Smith type and eruptive keratoacanthoma or Grzybowski type. Though solitary cutaneous keratoacanthomas are common, the multiple variants are extremely rare. A case of eruptive keratoacanthoma with oral involvement is presented with a brief discussion contrasting the two variants of multiple keratoacanthoma.

The role of human papillomavirus in the development of pyogenic granulomas

Background Pyogenic granulomas (lobular capillary hemangiomas) and condyloma acuminata share similar locations and risk factors. Human papillomavirus (HPV) types 6 and 11 are commonly associated with condyloma acuminata, but their association with pyogenic granulomas has not been evaluated. The purpose of this study was to determine whether pyogenic granulomas contain evidence of infection with condyloma producing HPVs.

Interaction between innate immunity and Ro52-induced antibody causes Sjögren's syndrome-like disorder in mice

Objectives Autoantibodies reactive with Ro52 are often found in sera of patients with Sjögrens syndrome (SS). This study was undertaken to investigate the role of Ro52-induced immune responses in pathogenesis of SS. Methods New Zealand Mixed (NZM) 2758 mice were immunised with Ro52 in alum adjuvant. Control mice were immunised either with maltose-binding protein or injected with alum alone. Mice were monitored for anti-Ro52 antibody, sialoadenitis and pilocarpine-induced salivation. Antibody binding to salivary gland (SG) cells was analysed in vivo and in vitro by immunofluorescence. Sera from immunised mice were passively transferred into untreated or alum injected NZM2758 mice. Results By day 30 post-immunisation, Ro52 immunised mice generated immunoprecipitating anti-Ro52 antibodies and they had the maximum drop in saliva production. Both Ro52 immunised and control mice showed evidence of mild sialoadenitis. However, only Ro52 immunised mice had antibody deposition in their SG. Passive transfer of Ro52-immune sera induced SG dysfunction in recipient mice, only if the recipients were primed with alum. In vitro, antibodies from Ro52-immune sera were internalised by a SG cell line and this uptake was inhibited by cytochalasin D treatment. Conclusions Our data show for the first time that antibodies induced by Ro52 are capable of inducing SG dysfunction, and that this phenomenon is dependent on the activation of innate immunity. The mouse model described in this study implies that autoantibody deposition in the SG might be an important step in the induction of xerostomia and pathogenesis of SS.