Lipoid proteinosis.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare, recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other tissues. It usually appears in infancy with hoarseness. We report a case of lipoid proteinosis in a 10-year-old boy that demonstrates the characteristic clinical, histologic, and ultramicroscopic features of this disease.

[Ultrastructural studies of Klebsiella rhinoscleromatis]. / Ul'trastrukturnye issledovaniia Klebsiella rhinoscleromatis.

In 1870, F. Hebra described a slowly progressing granulomatous disease of the nasal mucous membrane he called rhinoscleroma. Later, N. M. Volkovich and A. Frisch isolated from patients suffering from this disease a microorganism they termed Klebsiella rhinoscleromatis. In 1932, S. Belinov proposed to name the disease Scleroma respiratorium because the pathological process developing in rhinosclerosis may involve not only upper but also lower airways. In 1961, T. Steffen and J. Smith demonstrated that Klebsiella rhinoscleromatis conformed to Koch's postulates and was an etiological factor of inflammatory changes typical of scleroma. This paper presents results of ultrastructural examinations of Klebsiella rhinoscleromatis isolated from two patients with Scleroma respiratorium. It was shown that as the degenerative process developed, the amount of electron-dense material around the bacterial cell wall decreased progressively and only fragments of the cellular membrane remained.

Amyloid-like protein in children with rhinoscleroma.

Mucosal specimens from two children with rhinoscleroma have been investigated by light-, fluorescent- and electron microscopy. Amyloid-like protein was found, located not only in the vessel walls but also impregnated in the basic substance of the connective tissue. In this paper the genesis of this protein is discussed, assuming that it could be a result of auto-immune reactions.

M-cells in the epithelium of the nasopharyngeal tonsil.

Electron-microscope investigation on the epithelium was carried out in 23 nasopharyngeal tonsils. Adenoidectomy was performed on the ground of recurrent adenoiditis in the interattacks period in 13 boys and 10 girls between the ages of 2 to 4. The presence of five characteristic cellular types was found on the surface of the adenoid epithelium: tall cylindrical ciliated cells; cylindrical cells covered only with microvilli; secretory cells with the predominance of the so-called "dark granules"-producing cells; flat epithelial cells; cubic cells with microvilli on the surface and many vesicles in the cytoplasm. The last type of cells rank together with the M-cells, described by Owen and Jones in the epithelium of Peyer's patches in human beings and found in the palatal tonsil by various authors. A discussion is made on the pinocytary and secretory activity of the M-cells that is being linked with the immunogenesis of the nasopharyngeal tonsil. It is pointed out that in normal conditions the reticulation of the epithelium of the adenoid is found only in the zones that had their surface covered by M-cells.

Electron-microscope observations on the tonsillar epithelium in children with recurrent tonsillitis.

Twenty tonsils from children aged 3-6 years removed by operation due to recurrent tonsillitis have been examined. Electron-microscope observations of the epithelial layer have shown a lowering of the number and a reduction of the tubulo-vesicular system of the M-cells. The squamous stratified epithelium is predominating; it is keratinized in some places. Lymphocytes in an active phase and vascular activity in the parenchyma of the removed tonsils are very rare to observe; these were seen only in relation with the M-cells. The above-mentioned data may serve to prove Surjan Jr.'s 1980 hypothesis on the key-importance of metaplastic change of the tonsillar epithelium in recurrent tonsillitis pathogenesis.