Down syndrome is the most, well known autosomal trisomy and e large number of abnormal skin features have been reported to occur in patients with Down syndromir. including syringoma. Syringomas occur with increased frequency in patients with Down syringoma, usually limited to the region around the eyes. but few cases of eruptive syringoma with Down syndrome have been reported. We reported the case of familial eruptive syringoma in a 13 years old patient with Down syndrome.
Adolescent ; Down Syndrome* ; Humans ; Livedo Reticularis ; Skin ; Syringoma* ; Trisomy

BACKGROUND: Murine IL-2-induced lymphokine-activated killers can be divided into two mutually exclusive subset:NK1.1'CD8 and NK1.1 CD8+. However, there is a strong evidence that NK cell may belong to T cell lineage. Recently novel lymphocyte subsets, present in the adult murine thymus, CD3+NK1.1'TCRap(TNK) cell is readily identifiable in fresh obtained murine adult CD4 CD8 thymocytes. MATERIAL AND METHOD: We sorted out CD4 and CD8 (double negative.' DN) cells and CD8+ cells from murine spleen and cultivated these cells with IL-2. And the surface B220, CD8, NK1. 1 and cytopasmic NK1.1 was analysed simultaneously to see whether these cells can be switched to the other subtype of cells. RESULT: Purified DN cells were switched to several subtype of cells'. CD8'B220+(LAK cells), NK1.1'B220+(LAK cells), CD8 B220, cytoplasmic NK1.1+B220 cells. Purified CD8 cells were switched to CD8+B220' LAK cells and cytoplasmic NK1.1+ CD8+ B220+ and cytoplasmic NK1.1' CD8 B220 cells. In addition, the CD8' cells originated from DN cells do not express the cytoplasmic NK1.1 in contrary to the sorted CD8 cells. CONCLUSION: Our results indicated that these will be useful models to investigating CD8 precursor potentials in populations of CD4 CD8 (doble negative) cells and relationship of NK1.1 These results also supports the hypothesis that T cells and NK cells have same ontogeny and CD8 effector functions are potentially diverse and could be exploited by various conditions that switch off host protected cytolytic response. These model offer a way to study the molecular regulation of CD8 gene expression.
Adult ; Cell Lineage ; Cytoplasm ; Gene Expression ; Humans ; Interleukin-2* ; Interleukins* ; Killer Cells, Lymphokine-Activated ; Killer Cells, Natural ; Lymphocyte Subsets ; Spleen ; T-Lymphocytes ; Thymocytes ; Thymus Gland

Skin necrosis is a rare complication of heparin administration that is usually localized to injection sites. We report a case of skin necrosis that was caused by minidose intraarterial infusion of porcine heparin which had been used in a touch to prevent coagulation in percutaneous intraarterial cannula. The skin necrosis appeared 35 days after starting heparin use.
Catheters ; Heparin* ; Infusions, Intra-Arterial ; Necrosis* ; Skin

Hydroa Vacciniforme is a rare, chronic, photosensitivity disorder witli onset in childhood that is characterized by recurrent, discrete vesiculation, necrosis and vacciniform scarring limited to sun exposed skin. We report a case of hydroa vacciniforme associated with ocular inwlvment in which vesicles could be induced only with single MED exposure to UVA.
Cicatrix ; Hydroa Vacciniforme* ; Necrosis ; Photosensitivity Disorders ; Skin ; Solar System

Livedo vasculitis clinically shows purpuric papules and recurrent ulcers in the lower extremities, mainly on the ankles, leaving characteristic scars called atrophie blanche after the healing of the ulcers. Its characteristic histopathologic features and clinical evolution indicate that the common pathologic event is occlusion of vessels in the middle and deep dermis. In Korean literature, seven cases of this disease have been reported but the response of the treatment was not, satisfactory. We report three cases of livedo vasculitis cleared by combined ther by of acetylsalicylic acid, dipyridamole and nifedipine, which has not been reported in Korean literatur.
Ankle ; Aspirin* ; Cicatrix ; Dermis ; Dipyridamole* ; Lower Extremity ; Nifedipine* ; Ulcer ; Vasculitis*

BACKGROUND: Transforming growth factor-beta(TGF-beta) has multiple regulatory effects on cells of the immune system, and it has been suggested that differentiation of lymphoid cells is influenced by low concentra tions of this cytokine. OBJECTIVES: The aim or this study was to investigate the role of TGF-beta in regulation of T cell growth and differentiation, and to compare this effect with that of other cell signals known to be important in T cell ontogeny. METHODS: We used the CTLL-2 cell line in the presence of IL-2. Surface phenotype expression was analysed to see whether these cells could be switched to the other subtype of cells. RESULT: Treatment of CTLL-2 cells with TGF-beta resulted in dose dependent growth inhibition and morphological changes. Curing routine passage, less than 5% of cells were CD8alpha positive, whereas 38% of cells expressed CD8alpha when treated with IL-2 plus TGF-beta. However, TPA plus calcium ionophore, IFN-gamma, or TNF-alpha caused no significant changes in the proportion of CD8 cells. CONCLUSION: Our results show that this experiment can be a useful model for investigating CD8 precursor potentials in populations of CD4-CD8-(double negative) cells, and such a model may offer a way to study the molecular regulation of CD8 gene expression.
Calcium ; Cell Line* ; Gene Expression ; Immune System ; Interleukin-2 ; Lymphocytes ; Phenotype ; Transforming Growth Factor beta ; Tumor Necrosis Factor-alpha

BACKGROUND: Several reports have demonstrated that TGFalpha and EGF are mitogenic for keratinocytes. Whenther its expression on epithelial tumors is a marker of malignancy or signifies an important step in the development of neoplasia is poorly understood. EGF receptors are also present in normal epidermis and epithelial tumors but their physiological roles are not yet understood. OBJECTIVE: Our purpose was to examine the staining patterns of TGFalpha, EGF and EGF receptors on the npithelial tumors of the skin, and to investigate kinetics of expression of EGF receptors. METHODS: We performed immunoperoxidase staining(ABC technique) with monoclonal anti-TGFalpha antibody, polyclonal anti-EGF antibody and polyclonal anti-EGF receptor antibody on the formalinfixed, paraffin-embedded tissue specimens of benign, premalignant and malignant skin tumors. RESULTS: The density of the expression of TGFalpha and EGF was not correlated with the degree of the malignancy of the epithelial tumors and is neither constant in any kind of the tumors. However the infiltrative type of basal cell carcinoma(BCC) is stronger that its solid type on the expression of TGFalpha and EGF. All benign tumors demonstrated a diffuse pattern within tumor lobules. pression of TGFalpha and EGF. All benign tumors demonstrated a diffuse pattern within tumor lobules. Focal TGFalpha immunostaining was seen in three of 10 squamous cell carcinomas(SCC) and four of 10 BCCs. TGFalpha immunostaining was absent from the outermost one to two layers of tumor lobules of all keratoacanthomas. The specimens which increased the expression of TGFalpha and EGF tended to decrease the expression of EGF receptor. CONCLUSION: These data suggest that the density of immunohistochemical expression of TGFalpha and EGF may be not dependent on the differentiation of tumor cells, and the pattern of immunohistochemical expression of TGFalpha can differentiate SCC from benign tumors such as keratoacanthoma. FGF receptor may be occupied by both of TGFalpha and EGF. With the receptors being occupied, a down regulation of the receptors may occur which results in decreased EGF receptor expression.
Down-Regulation ; Epidermal Growth Factor* ; Epidermis ; Keratinocytes ; Keratoacanthoma ; Kinetics ; Receptor, Epidermal Growth Factor* ; Receptors, Fibroblast Growth Factor ; Skin* ; Transforming Growth Factor alpha*

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.
Actins ; Desmin ; Female ; Fibroblasts ; Follow-Up Studies ; Humans ; Infant, Newborn ; Myocytes, Smooth Muscle ; Myofibroblasts ; Myofibromatosis* ; Necrosis ; Parturition ; Pericytes ; Thorax

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.
Antibodies ; Antibodies, Monoclonal ; Biotin ; Bowen's Disease ; Coloring Agents ; Diagnosis ; Melanoma ; Molecular Weight ; Skin ; Streptavidin

We report two cases of linear focal elastosis. They are a 16-year-old girl and a 14-year-old boy who had had asymptomatic, several, yellow-red, slightly elevated, palpable, striae-like lesions on their middle and lower back since their early childhood. Light microscopic examinations of skin biopsy specimens demonstrated dermal thickening and focal increases of thin wavy fibers in the upper and mid-dermis but no changes in the epidermis. Verhoeff-van Gieson stains for elastic fiber revealed aggregated, clumped, curled, or fragmented elastic fibers.
Adolescent ; Biopsy ; Coloring Agents ; Elastic Tissue ; Epidermis ; Female ; Humans ; Male ; Skin