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 41 year-old man had dark pigmented papules and plaques, 4 to 15 mm in size, on the proximal portion of the dorsum of the penile shaft and pubic area of 8 months duration. Over next several months, flesh colored, macerated papules, 2 to 4 mm in size, appeared on the left side of the scrotum Light microscopic examination from a pigmented lesion of the penile shaft showed typical findings of Bowen's disease. Ultrastructural findings included: widening of intercellular space, a decrease in the number of desmosomes with microvillous projections, aggregation of tonofilaments, an increase in the mitotic keratinocytes, an increase in the number of melanin granules, and disorganized bundles of tonofilaments surrounding chromosomes. Virus-like particles were not observed. Based on the ultrastructural findings and review of literatures, it seems to be probable that bowenoid papulosis is a clinical variant of Bowens disease.
Adult ; Bowen's Disease ; Desmosomes ; Extracellular Space ; Genitalia* ; Humans ; Intermediate Filaments ; Keratinocytes ; Melanins ; Scrotum

Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.
Blister ; Dermatitis, Exfoliative ; Epidermis ; Humans ; Ichthyosis Bullosa of Siemens* ; Ichthyosis* ; Intermediate Filaments ; Molting ; Skin ; Wills

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

BACKGROUND: In the course of the study of keratin expression in the epidermis of nevus sebaceus, several cells in the epidermis of nevus sebaceus were positively stained with CAM 5.2 antibody, which is known to be specific for the lower molecular weight cytokeratin and used as a marker of Merkel cell. OBJECTIVE: This study was intended to verify that CAM 5.2 positive cells found in the epidermis of nevus sebaceus are Merkel cells and to understand the meaning of CAM 5.2 positive j cells in the epidermis of nevus sebaceus. METHODS: The immunohistochemical stainings with CAM 5.2 and antibody to epithelial membrane antigen (EMA) performed on specimens of normal skin, epidermal nevus, nevus sebaceus and some appendage tumors. In order to confirm the nature of CAM 5.2 positive cells, the distribution of those were compared to that of Merkel cells and double labeling with CAM 5.2 and neurofilament was performed. RESULTS: CAM 5.2 positive cells were also found in trichilemmoma developed associated with nevus sebaceus and the epidermis of normal paimoplantar skin. CAM 5.2 positive cells were also stained with antibody to EMA on serial sections cut from the same tissue blocks. The association of CAM 5.2 positive cell and nerve fiber was also demonstrated. CONCLUSION: CAM 5.2 positive cells are seemed to be Merkel cells and their presence in the covering epidermis of nevus sebaceus suggests to the epidermis of nevus sebaceus may not be nevoid proliferation of epidermal keratinocytes.
Epidermis* ; Intermediate Filaments ; Keratinocytes ; Keratins ; Merkel Cells ; Molecular Weight ; Mucin-1 ; Nerve Fibers ; Nevus* ; Skin

BACKGROUND: In the course of the study of keratin expression in the epidermis of nevus sebaceus, several cells in the epidermis of nevus sebaceus were positively stained with CAM 5.2 antibody, which is known to be specific for the lower molecular weight cytokeratin and used as a marker of Merkel cell. OBJECTIVE: This study was intended to verify that CAM 5.2 positive cells found in the epidermis of nevus sebaceus are Merkel cells and to understand the meaning of CAM 5.2 positive j cells in the epidermis of nevus sebaceus. METHODS: The immunohistochemical stainings with CAM 5.2 and antibody to epithelial membrane antigen (EMA) performed on specimens of normal skin, epidermal nevus, nevus sebaceus and some appendage tumors. In order to confirm the nature of CAM 5.2 positive cells, the distribution of those were compared to that of Merkel cells and double labeling with CAM 5.2 and neurofilament was performed. RESULTS: CAM 5.2 positive cells were also found in trichilemmoma developed associated with nevus sebaceus and the epidermis of normal paimoplantar skin. CAM 5.2 positive cells were also stained with antibody to EMA on serial sections cut from the same tissue blocks. The association of CAM 5.2 positive cell and nerve fiber was also demonstrated. CONCLUSION: CAM 5.2 positive cells are seemed to be Merkel cells and their presence in the covering epidermis of nevus sebaceus suggests to the epidermis of nevus sebaceus may not be nevoid proliferation of epidermal keratinocytes.
Epidermis* ; Intermediate Filaments ; Keratinocytes ; Keratins ; Merkel Cells ; Molecular Weight ; Mucin-1 ; Nerve Fibers ; Nevus* ; Skin

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*

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

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