We report a case of focal dermal hypoplasia associated with ichthyosis in an 18-year-old Korean female. The patient showed a yellowish atrophic patch on the left side of her abdomen, abnormal skin pigmentation, nail dystrophy and ichthyosis since birth.We could also find skeletal and dental deformities. The biopsy specimen obtained from the yellowish atrophic patch showed marked attenuation of collagen fibers and ectopic fatty tissue nearly reaching up to the upper dermis.
Abdomen ; Adipose Tissue ; Adolescent ; Biopsy ; Collagen ; Congenital Abnormalities ; Dermis ; Female ; Focal Dermal Hypoplasia* ; Humans ; Ichthyosis* ; Skin Pigmentation

We report a case of focal dermal hypoplasia associated with ichthyosis in an 18-year-old Korean female. The patient showed a yellowish atrophic patch on the left side of her abdomen, abnormal skin pigmentation, nail dystrophy and ichthyosis since birth.We could also find skeletal and dental deformities. The biopsy specimen obtained from the yellowish atrophic patch showed marked attenuation of collagen fibers and ectopic fatty tissue nearly reaching up to the upper dermis.
Abdomen ; Adipose Tissue ; Adolescent ; Biopsy ; Collagen ; Congenital Abnormalities ; Dermis ; Female ; Focal Dermal Hypoplasia* ; Humans ; Ichthyosis* ; Skin Pigmentation

The clinicolaboratory findings, cardiac catheterization, and outcome of operation were analyzed in 78 patients with tetralogy of Fallot, which were diagnosed by echocardiography, cardiac catheterization, and a cardiac angiography and confirmed by operation at Dong San Hospital, Keimyung University, during a 4(1/2)-year period from January 1984 to June 1988. The following results were obtained : 1) Out of the 78 cases, 45 were male and 64 were female. 2) The electrocardiogram showed right axis deviation in 71 cases(91%) and right ventricular hypertrophy in 66 cases(84.6%). 3) Chest X-ray revealed that, the cardiothoracic ratio was normal or decreased in 68 cases(87.3%), and cardiac apex elevation was noticed in 57 cases(73%). 4) Pulmonary stenosis were chiefly valvular and infundibular type(53.8%). 5) The associated heart diseases with TOF were patent foramen ovale(64.1%), right sided aortic arch(19.2%), and secundum ASD(10.3%), in that order. 6) There was an intimate correlation between secondary polycythemia and thrombocytopenia. 7) Among the 78 Cases, 73 cases had total correction, 1 case had shunt operation, and 4cases had total correction after shunt operation. The highest mortality rate occurred in the cases of total correction after shunt operation(25%). The cases with a main pulmonary artery size of 1/3 to 2/3 against, the aorta had a higher mortality than the other group, and no cases expired in the group with a ratio of more than 2/3. The overall surgical mortality was 8.9%.
Angiography ; Aorta ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography ; Electrocardiography ; Female ; Heart Diseases ; Humans ; Hypertrophy, Right Ventricular ; Male ; Mortality ; Polycythemia ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Tetralogy of Fallot* ; Thorax ; Thrombocytopenia

The authors experience 3 cases of angiographically occult vascular malformation(AVOM) of the brain during the last 3 months in 1990. All cases were diagnosis of either operative or pathological finding and were associated with intracerebral hemorrhage. The authors recommend that surgery should be considered even to those angiographically negative intracerebral hemorrhage cases, once clinical features are highly suggestive of AOVM. Surgery can be an effective and define therapy for these entities, which are prone to cause recurrent hemorrhage and persistent neural deficits.
Brain* ; Cerebral Hemorrhage ; Diagnosis ; Hemorrhage ; Magnetic Resonance Imaging ; Vascular Malformations*

No abstract available.
Animals ; Cell Adhesion* ; Female ; Leiomyoma* ; Mice ; Myometrium* ; Neural Cell Adhesion Molecules*

Ultrasonograms of proven liver abscesses in 44 patients were analysed. In ulltrasonography prior to otherdiagnostic approach, 38 patients(86.4%) were accurately diagnosed as liver abscess with correlation of clinicalsymptoms. The outline of abscess is smoothly defined. oval and round in 19 patients, irregularly and ill-definedin 25 patients. Only in 5 patients echogenic capsules are demonstrated. The internal echogenecity is variable;they are predominantly echo-free in 14, predominantly low-echo in 25 and predominantly echogenic, similar to solidtumor in 2 patients . 36 patients who were treated by medication only and combined needle aspiration had follow-up ultrasonography within 3 weeks since initial treatment. 33 patients were ultrasonically and clinically improved.Only one patients died of sepsis after needle aspiration and 2 patients were discharged without any sign ofimprovement.
Abscess ; Capsules ; Follow-Up Studies ; Humans ; Liver Abscess ; Liver ; Needles ; Sepsis ; Ultrasonography

Generation of various autoantibodies and associated autoimmune diseases has been reported in allogeneic and autologous stem cell transplant recipients. We report a case of scleroderma following autologous peripheral blood stem cell transplantation.
Autoantibodies ; Autoimmune Diseases ; Peripheral Blood Stem Cell Transplantation* ; Stem Cells ; Transplantation

BACKGROUND: Pemphigus vulgaris is an autoimmune bullous dermatoses of skin and mucosa characterized by loss of adhesion between keratinocytes, a process known as acantholysis. Apoptosis, programmed cell death, may participate in pathogenesis of intercellular detachment and loss of cell-matrix interaction. OBJECTIVE: This study was designed to investigate the induction of apoptosis in the pemphigus lesional epidermis and to elucidate the mechanism of apoptosis induced by pemphigus sera. METHODS: Hoechst 33342 staining was performed to determine the induction of apoptosis in the pemphigus lesional epidermis. In addition, we used HaCaT cells treated with pemphigus sera and analyzed the expression of caspase-3, caspase-8, caspase-9 and bcl-2, bcl-xL, bax, bak by the RT-PCR method. RESULTS: In Hoechst 33342 staining, typical findings of apoptosis were observed in the pemphigus lesional epidermis showing acantholysis. RT-PCR showed the upregulation of caspase group (caspase-3, caspase-8, caspase-9), the downregulation of antiapoptotic bcl-2 family (bcl-2, bcl-xL) and the upregulation of proapoptotic bcl-2 family (bax, bak). CONCLUSION: These results suggest that apoptosis may be associated with acantholysis of pemphigus lesional epidermis and may play an important role in the pathogenesis of pemphigus.
Acantholysis ; Apoptosis* ; Caspase 3 ; Caspase 8 ; Caspase 9 ; Cell Death ; Down-Regulation ; Epidermis ; Humans ; Keratinocytes ; Mucous Membrane ; Pemphigus* ; Skin ; Skin Diseases, Vesiculobullous ; Up-Regulation

Laugier-Hunziker syndrome (LHS) is a rare acquired benign macular hyperpigmentation of the lips and oral mucosa which is often associated with longitudinal melanonychia. LHS is known to be an entirely benign condition with no systemic manifestations, which requires patient reassurance as the only intervention. The significance of this condition is due to its inclusion in differential diagnoses of pigmentary disorders of the oral mucosa, especially Peutz-Jeghers syndrome. We report a case of Laugier-Hunziker syndrome in a 66-year-old woman who presented with longitudinal pigmented bands on her fingernails and multiple, pigmented macules on the lip and tongue.
Aged ; Diagnosis, Differential ; Female ; Humans ; Hyperpigmentation ; Lip ; Mouth Mucosa ; Nails ; Peutz-Jeghers Syndrome ; Tongue

Tattooing and other forms of body art have recently become popular fashion accessories worldwide. Temporary henna tattooing has also become increasingly popular and is readily available. Henna, which is called Mehndi in Indian, is a plant that is commonly used as a temporary dyeing agent. Para-phenylenediamine (PPD) is added to the Henna tattooing process in most cases to hasten the drying process and enhance darkness. Since PPD is widely recognized as a sensitizer, the risk of allergic contact dermatitis from henna tattoo mixtures is increasing. We report a case of contact dermatitis from a henna tattoo mixture, which developed 2 weeks after henna tattooing.
Darkness ; Dermatitis, Allergic Contact* ; Dermatitis, Contact ; Plants ; Tattooing*