The Dowling-Meara type of epidermolysis bullosa simplex(EBS) is genetic disease that is transmitted as an autosomal dominant trait and is charscterized clinically by pherpetiform clustering of blisters and palmo-plantar keratoderma. The disease usually presents at with or in early infancy. Although serious and hemorrhagic Wers may occur on any part of the body, the lesions heal without scaning in general. The disease shows a tendency to improve by progressian of age and it usually follows a relatively benign course. Microecopically, there are intraepidermal bli.ter s forming as a result of cytolysis of basal cells. In addition, the is a highly characteristic clumping of tonofilaments of keratinocytes in the lower epidermis, which is not seen in any other form of EBS. We report a case of Dowling-Meara type of EBS that is first destribed in Korean medical literatures.
Blister ; Epidermis ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Intermediate Filaments ; Keratinocytes

No abstract available.
Desmin* ; Intermediate Filament Proteins* ; Intermediate Filaments* ; Muscle, Skeletal* ; Muscular Diseases* ; Sciatic Nerve* ; Vimentin*

A 25-year-old man with familial benign chronic pemphigus presented with a one-year history of a localized pruritic recurrent eruption on his perianal area, Physical examination showed moist, macerated, fissured and scaly patches on erythematous base, A biopsy specimen showed extensive intraepidermal separation containing acantholytic cells. Electron microscopic observation showed widened intercellular space, detachment of the tonofilaments from the desmosomes, and subsequent concentration of the tono filaments around the nucleus. Microvilli were elorigated, thinned and branehed. 1)esmosomes were reduced in number. This case is unique in its clinical location, showing no family tendency.
Adult ; Biopsy ; Desmosomes ; Extracellular Space ; Humans ; Intermediate Filaments ; Microvilli ; Pemphigus, Benign Familial* ; Physical Examination

BACKGROUND: The histologic distinction between basal cell carcinoma(BCC) and squamous cell carcinoma(SCC) is sometimes difficult, but clinically important, because SCC has worse prognosis than BCC. Cytokeratins(CKs) are the major component of intermediate filaments and are subdivided into at least 20 different polypeptides. Monoclonal antibodies developed against these individual keratins become very useful in the classification of the major types of epithelial tumors. OBJECTIVE: The purpose was to investigate the usefulness of CK staining in distinguishing BCC from SCC. METHODS: We studied 10 cases of BCC and 10 cases of SCC with 6 anticytokeratin antibodies including AE1, CAM5.2, CK7, CK16, CK10, and CK8. RESULTS: All cases of BCC and SCC stained with AE1. Six and 5 cases out of 10 cases of BCC stained with CAM5,2 and CK7, respectively, but all cases of SCC stained with neither CAM5.2 nor CK7. All cases of SCC stained with CK16, but all cases of BCC did not. Two out of 10 cases of SCC stained focally with CK10, while all cases of BCC did not stain with CK10. All cases of BCC and SCC did not stain with CK8. CONCLUSION: We conclude that a panel of antibodies for CKs including CK7, CAM5.2, and CK16 may be useful in distinguishing BCC from SCC. In particular, CK16 may be the most useful marker because it was positive for all cases of SCC while negative for all cases of BCC.
Antibodies ; Antibodies, Monoclonal ; Carcinoma, Basal Cell* ; Carcinoma, Squamous Cell* ; Classification ; Intermediate Filaments ; Keratins* ; Peptides ; Prognosis

Metastasis is one of hallmarks of cancer and a major cause of cancer death. Combatting metastasis is highly challenging. To overcome these difficulties, researchers have focused on physical properties of metastatic cancer cells. Metastatic cancer cells from patients are softer than benign cancer or normal cells. Changes of viscoelasticity of cancer cells are related to the keratin network. Unexpectedly, keratin network is dynamic and regulation of keratin network is important to the metastasis of cancer. Keratin is composed of heteropolymer of type I and II. Keratin connects from the plasma membrane to nucleus. Several proteins including kinases, and protein phosphatases bind to keratin intermediate filaments. Several endogenous compounds or toxic compounds induce phosphorylation and reorganization of keratin network in cancer cells, leading to increased migration. Continuous phosphorylation of keratin results in loss of keratin, which is one of the features of epithelial mesenchymal transition (EMT). Therefore, several proteins involved in phosphorylation and reorganization of keratin also have a role in EMT. It is likely that compounds controlling phosphorylation and reorganization of keratin are potential candidates for combating EMT and metastasis.
Carcinogenesis* ; Cell Membrane ; Epithelial-Mesenchymal Transition* ; Humans ; Intermediate Filaments ; Neoplasm Metastasis ; Phosphoprotein Phosphatases ; Phosphorylation* ; Phosphotransferases

The stiffness of cancer cells is attributable to intermediate filaments such as keratin. Perinuclear reorganization via phosphorylation of specific serine residue in keratin is implicated in the deformability of metastatic cancer cells including the human pancreatic carcinoma cell line (PANC-1). 12-O-Tetradecanoylphorbol-13-acetate (TPA) is a potent tumor promoter and protein kinase C (PKC) activator. However, its effects on phosphorylation and reorganization of keratin 8 (K8) are not well known. Therefore, we examined the underlying mechanism and effect of TPA on K8 phosphorylation and reorganization. TPA induced phosphorylation and reorganization of K8 and transglutaminase-2 (Tgase-2) expression in a time- and dose-dependent manner in PANC-1 cells. These effects peaked after 45 min and 100 nM of TPA treatment. We next investigated, using cystamine (CTM), Tgase inhibitor, and Tgase-2 gene silencing, Tgase-2's possible involvement in TPA-induced K8 phosphorylation and reorganization. We found that Tgase-2 gene silencing inhibited K8 phosphorylation and reorganization in PANC-1 cells. Tgase-2 gene silencing, we additionally discovered, suppressed TPA-induced migration of PANC-1 cells and Tgase-2 overexpression induced migration of PANC-1 cells. Overall, these results suggested that TPA induced K8 phosphorylation and reorganization via Tgase-2 expression in PANC-1 cells.
Cell Line ; Cystamine ; Gene Silencing ; Humans ; Intermediate Filaments ; Keratin-8* ; Phosphorylation* ; Protein Kinase C ; Serine

Osteofibrous dysplasia (OFD)-like adamantinoma is a rare skeletal tumor that is characterized by the predominant OFD-like pattern with scattered epithelial nests. Adamantinoma shares clinical features (the majority of lesions in the tibia and the prevalent age group), radiologic findings (radiolucency with sclerotic shadow), and pathologic similarities (particularly the presence of scattered cytokeratin-positive stromal cells) with OFD. We describe a case of OFD-like adamantinoma. Epithelial cell nests express the epithelial membrane antigen, pancytokeratin, CK14, and collagen type IV. Ultrastructurally, the oval to spindle cells in the epithelial foci had abundant tonofilaments, and well-formed desmosomes with dense plaques, of which well preserved desmosomes are demonstrated for the first time in OFD-like adamantinoma. These immunohistochemical and ultrastructural findings further support that the origin of epithelial cells of classic and OFD-like adamantinoma are epithelial cells transformed from fibroblastic cells in the proliferating osteofibrous tissue.
Adamantinoma* ; Collagen Type IV ; Desmosomes ; Epithelial Cells ; Fibroblasts ; Fibroma, Ossifying ; Immunohistochemistry ; Intermediate Filaments ; Mucin-1 ; Tibia

The purpose of this study was to investigate what the intermediate filaments in the cells of rat mandibular fossa fibrous layer are and any relationships between the presence of these filaments and aging. Mandibular fossae of 4 groups of rats(14-day, 28-day, 55-day and adult groups) were removed on bloc and processed for immunostaining and were subjected to light microscopic examination. The results were as follows : In 14-day group, there were no immunoreactive cells in fibrous layer of mandibular condyle articular surface. But in 28-day group, many immunoreactive cells were seen in fibrous layer, especially central portion of articular surface of mandibular fossa. These cells were fusiform shaped and immunoreactivities were seen in the cytoplasm around the nucleus. In 55-day group many immunoreactive cells were seen in fibrous layer of mandibular fossa. These cells were fusiform shaped and distributed evenly in central portion of this fibrous layer. Immunoreactivities were seen in the cytoplasm around the nucleus. In adult group, the results were similar to 55-day group, Many immunoreactive cells were seen in fibrous layer of mandibular fossa especially central portion. According to these results, vimentin immunoreactive filaments appear with aging and increment of mechanical load associated with incision or mastication.
Adult ; Aging* ; Animals ; Cytoplasm ; Humans ; Intermediate Filaments ; Mandibular Condyle ; Mastication ; Rats* ; Vimentin*

Ameloblastoma is the most common odontogenic tumor of the jawbones, but the origin of this tumor has been remained to be unproven. Cytokeratins (CKs) are specific intermediate filament of epithelial cells, and vimentin is expressed in mesenchymal cells. The immunohistochemical detection of different CKs and vimentin has made it easier to know the origin of tumor. Paraffin-embedded tissue sections from 15 ameloblastomas and 1 ameloblastic carcinoma were used for immunohistochemical evaluation of CK 7, 8, 13, 14, 19 and vimentin. Their expression is evaluated in different tumor cells, which are observed in different type of tumors. In the follicular and reticular subtype, central stellate cells of tumor nests expressed CK 8, 14, 19 and peripheral columnar cells expressed CK 14. CK 7, and 13 were not expressed. Vimentin was detected in fibrous stroma around tumor nest, not in tumor cells. The tumor cells of ameloblastic carcinoma expressed CK 7, 14 and 19, but CK 8 was more weakly stained than that in ameloblastoma. Central stellate cells and peripheral columnar cells of acanthomatous subtype showed same expression pattern with others. Meta plastic squamous cells expressed CK 8, 14, 19 and keratinizing squamous cells expressed CK 13, 19. CK 7 and vimentin were not detected in tumor cells and vimentin was expressed in fibrous stroma. Most of the tumor cells of ameloblastoma showed CK 14 and CK 19 and did not express CK 7 and vimentin. These findings were similar to the immunophenotype of dental lamina. And these results will be beneficial to differential diagnosis of odontogenic tumors and other kind of tumors arising at the oral cavity.
Ameloblastoma* ; Ameloblasts ; Diagnosis, Differential ; Epithelial Cells ; Intermediate Filaments ; Keratins* ; Mouth ; Odontogenic Tumors ; Plastics ; Vimentin*

Epidermolytic keratosis palmaris is a rare disease which shows clinical findings of Unna Thost keratoderma and histopathologic of epidermolytic hyperkeratosis. We report herein a case of epidermolytic keratosis palmaris et plantaris in a 16-month-old female baby. Light microscopy shows marked hyperkeratosis, large irregular keratohyalin granules, and large clear spaces in the granular and upper spinous layers. Eletron microscopic findings shows that the clear spaces are areas of cytoplasm filled with a fibrillar material and cellular organelles. Abnormal clumping of tonofilament and keratohyalin is also present.
Cytoplasm ; Female ; Humans ; Hyperkeratosis, Epidermolytic ; Infant ; Intermediate Filaments ; Keratoderma, Palmoplantar* ; Keratosis* ; Microscopy ; Organelles ; Rare Diseases