Dyke-Davidoff-Masson syndrome is a rare clinical syndrome consisting of hemiatrophy of cerebral hemisphere, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. Its clinical manifestations are hemiparesis, several types of seizure, mental retardation and other neurologic deficits. We experienced a case of Dyke-Davidoff-Masson syndrome in a four months old boy who visited to our department due to right side upper extremity weakness and infantile spasm. The brain magnetic resonance imaging showed marked hemiatrophy of left cerebral hemisphere and enlargement of left lateral ventricle. So with the review of literatures, we report a case of Dyke-Davidoff-Masson syndrome with infantile spasm.
Brain ; Cerebrum ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Lateral Ventricles ; Magnetic Resonance Imaging ; Male ; Neurologic Manifestations ; Paresis ; Petrous Bone ; Seizures ; Spasms, Infantile* ; Temporal Bone ; Upper Extremity

No abstract available.

BACKGROUND: Calcinosis cutis may occur when connective tissue is abnormal (dystrophic), or where calcium or phosphate levels in the blood are high(metastatic); alternatively, there may be no obvious underlying cause(idiopathic). The exact incidence of calcinosis cutis in dermatologic patients is not well-kniown and the pathomechanism of it remains to be elucidated. OBJECTIVE: This study was performed to analyze the incidence, clinical and histopathological features of calcinosis cutis. METHODS: We studied 72 patients with calcinosis cutis who visited our department between January 1985 and December 1996. The patients were classified into 3 types (dystrophic, metastatic, and idiopathic) and were analyzed clinically and histopathologically. Results : The results were summerized as follows; 1. There were 60 cases(83.3%) and 12 cases(16.7%) of the dystrophic and idiopathic types, respectively but the metastatic type was not observed. The frequency of dystrophic calcification in individual disorders was 88.2% in pseudoxanthoma elasticum, 66.7% in trichilemmal cyst and dermatomyositis, and 64.6% in pilomatrichoma. 2. In the idiopathic type, it was common in females aged over 50 years and the mean duration of lesions was 2.8+/-2.0 years. The predilection site was the flank(5 cases) and most of the lesions(7 cases) showed skin-colored nodules 3. Histopathologically, calcified material of the idiopathic type was located predominently in the dermis(10 cases) and was present as large masses surrounded by a foreign body reaction. 4. In the dystrophic type, calcified deposits were noted predominently within the shadow cells of pilomatricoma, in the degenerated elastic fibers of pseudoxanthoma elasticum, and in the keratinized area of basal cell carcinoma, squamous cell carcinoma, trichilemmal cyst, and epidermal cyst. Foreign body reactions with giant cells and mononuclear cell infiltrations were often found around large deposits of calcium. Conclusion : The incidence of calcinosis cutis in dermatologic patients is relatively low, but a variety of disorders can be associated with cutaneous calcification. Therefore, dermatologists should be familiar with the different forms of cutaneous calcification and the dermatoses that manifest them.
Calcinosis* ; Calcium ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Connective Tissue ; Dermatomyositis ; Elastic Tissue ; Epidermal Cyst ; Female ; Foreign Bodies ; Foreign-Body Reaction ; Giant Cells ; Humans ; Incidence ; Pilomatrixoma ; Pseudoxanthoma Elasticum ; Skin Diseases

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

Multiple endocrine neoplasi~a type 3 is a rare, heritable or sporadic, multiple neoplastic disorder that is charracterized by thyroid medullary careinoma, pheochromocytoma, multiple mucosal neuroma, and marfanoid habitus. The most important disorder of the syndrome is mucosal neuroma that is an early diagnostic sign of multiple endocrine neoplasia type 3. Early diagnosis of MEN type 3 determines prognosis of the disease. We present a case of multiple endocrine neoplasia type 3 of the sporadic pattern in a 27-year-old man who had typical medullary thyroid carcinoma, mucosal neuroma, marfanoid habitus, and megacolon.
Adult ; Early Diagnosis ; Humans ; Male ; Megacolon ; Multiple Endocrine Neoplasia* ; Neuroma* ; Pheochromocytoma ; Prognosis ; Thyroid Gland ; Thyroid Neoplasms

No abstract available.
Dimethylformamide* ; Liver Diseases* ; Liver*

BACKGROUND: The pathogenesis of confluent and reticulated papillomatosis(CRP) is still unknown, although many theories have been suggested. It is stressed that abnormal host response to colonization of pityrosporum orbiculare might play a role in the pathogenesis of CRP, but is not completely understood. Frequently, we have observed bacterial colonies in the stratum corneum and, especially, within the hair follicles from biopsy specimens of patients with CRP. In addition, successful treatment for CRP with minocycline, a derivative of tetracyclines, has been reported continuously. OBJECTIVE: The purpose of this study was to document the possible association of bacterial factors in the pathogenesis of CRP and to elucidate the effectiveness of minocycline. METHODS: We performed Brown and Brenn gram staining in 12 biopsy specimens, bacterial cultures, identification of microorganisms and antibiotics sensitivity testing including minocycline. We administered oral minocycline with an initial dose of 100mg per day for 1 to 3 months and a maintenance dose 50mg per day for I to 2 months, then evaluated the response of treatment. RESULTS: 1. On Brown and Brenn staining, the gram positive bacterial colonies that stained dark bluish or nearly black were observed within the infundibulum of hair follicles in 10 out of 12 biopsy specimens(83.3%) and on the keratotic invagination of stratum corneum in 11 out of 12 biopsy specimens(91.7%). 2. Histological features of regions where bacterial colonies were observed showed, hyperkeratosis and keratotic invagination on the stratum corneum in all cases. Hyperkeratosis(66.7%), parakeratosis(16.7%), inflammatory cell infiltration(25.0%), perifollicular fibrosis(33.3%), and abnormal keratin in sebaceous ducts(50.0%) were shown in hair follicles. 3. In anaerobic cultures, no bacteria was grown. In aerobic cultures, staphylococcus species were identified in 6 cases, streptococcus viridans in one case, sternotrophomonas maltophilia in one case. In the susceptibiliity test, minocycline was sensitive in 7 from 8 cases. 4. The therapeutic response to minocycline was observed within one month in all cases, and the time to clear the lesions was 1/2 month in 2 cases, 1 month in 4 cases, 2 months in 5 cases, 3 months in one case, respectively. CONCLUSION: Form these results, we suggest that bacterial factors may play an important role in the pathogenesis of CRP and that the action mechanism of minocycline in the treatment of CRP may be due to antibacterial effects.
Anti-Bacterial Agents ; Bacteria* ; Biopsy ; Colon ; Hair Follicle ; Humans ; Malassezia ; Minocycline ; Papilloma* ; Staphylococcus ; Tetracyclines ; Viridans Streptococci

BACKGROUND: It seems that herpes zoster is caused by reactivation of the varicella-zoster virus and its incidence is increasing. The reactivation of the varicella zoster virus is thought to be associated with the disturbance of the state of immunity in patients with herpes zoster. OBJECTIVE: The purpose of this study was to elucidate the state of immunity in patients with herpes zoster in its acute phase(less than 7 days). METHODS: 1. Thirty patients with acute phase herpes zoster matched by age and sex against a control group, were checked for Helper/Inducer T cell(CD4), Suppressor/Cytotoxic T cell(CD8), NK cell, B cell and activated T cell by three color flow cytometric analysis. 2. Forty patients with herpes zoster measured delayed cutaneous hypersensitivity by means of Multitest' CMI. 3. Thirty patients with herpes zoster measured Ig G, M, A by means of N-antisera method.
Herpes Zoster* ; Herpesvirus 3, Human ; Humans ; Hypersensitivity ; Immunity, Cellular ; Immunity, Humoral* ; Incidence ; Killer Cells, Natural

BACKGROUND: Mycosis fungoides(MF) is a form of cutaneous T cell lymphoma with clonal differentiation of helpr' T cell. It has a patch, plaque, and tumor stage. But pathogenetic factors controlling the development and progression of MF are still unclear. Apoptosis plays a major role in developmental biology and homeostasis. The bcl-2 oncogene prolongs ce11 life by inhibiting apoptosis. The mutant pS3 gene induces apoptosis indirectly. Ki-67 antigen is the cell proliferation marker. Recently, it has been shown that the relationships among them are important in the tumorigenesis of the various tumors. OBJECTIVE: The aim of this study was to examine the expression of these genes and apoptotic rate and clarify the relationship among them in the development and progression of MF. METHODS: The eighteen specimens from 8 patients with MF and 10 specimens from benign lymphocytic infiltrating diseases including 5 lichen planus, 3 lupus erythematosus, and 2 contact dermatitis were included. We performed immunoperoxidase staining(LSAB technique) using monoclonal antibodies including bc1-2, p~53, and Ki-67(MIB1). We used ApoptaqTM(Oncor) in situ labelling kit for detecting apoptotic cell.
Antibodies, Monoclonal ; Apoptosis* ; Carcinogenesis ; Cell Proliferation ; Dermatitis, Contact ; Developmental Biology ; Homeostasis ; Humans ; Ki-67 Antigen ; Lichen Planus ; Lymphoma, T-Cell, Cutaneous ; Mycosis Fungoides* ; Oncogenes

No abstract available.
Candida albicans* ; Candida*