Letterer-Siwe disease is a non-lipid reticuloendotheliosis of unknown etiology and usually grouped with Hand-Schuller-Christian disease and eosinophilic granuloma as components of histiocytosis-X. It occurs mostly in infancy and, clinically it is the most generalliaed and serious. In recent years, the prognosis of infants with Letterer-Siwe disease has improved, primarily due to more aggressive therapy with corticosteroids and antimetabolic agents. The 14 months old male patient was admitted into the Severance Hospital on 15th, July 1974 for evaluation of a chronic and refractory skin eruptions, abdominal distension with diarrhea and edema on extremities. He had been well until 3 months old age when he developed seborrhea-like skin eruptions which did not improve on treatment for seborrhea. Vigorous treatment was started for the baby from the beginning of admission day. Routine laboratory tests, skin biopsy, bone marrow aspiration and radiologic studies for bony structures wer done. All those studies had showed severe anemia, thrombocytopenia, multiple bony defects in skull and numerous non-lipid histiocytic infiltration in skin and bone marrow all those were consist with Letterer-Siwe disease.
Adrenal Cortex Hormones ; Anemia ; Biopsy ; Bone Marrow ; Dermatitis, Seborrheic ; Diarrhea ; Edema ; Eosinophilic Granuloma ; Extremities ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Male ; Prognosis ; Skin ; Skin Tests ; Skull ; Thrombocytopenia

Gestational trophoblastic disease associated with the tubal pregnancy is uncommon, and the incidence has been described as 1/5000 tubal pregnancy. We have experienced a case of metastatic gestational trophoblastic disease(GTD) in the lung occuring with complete hydatidiform mole arising in tubal pregnancy. The patient was a 39-year-old, G4P2A2L2 woman with amenorrhea for 5 weeks. Ectopic pregnancy in the right fallopian tube was suspected on transvaginal ultrasonogram. A right adnexectomy was performed. The fallopian tube was markedly dilated and ruptured. The right ovary and a round hematoma had adhered to the external surface of the fallopian tube. On gross examination, no molar tissue was identified. On microscopic examination, the lumen of the dilated fallopian tube was filled with blood clots admixed with several chorionic villi showing hydropic swelling and marked proliferation of atypical trophoblasts. Proliferating syncytio-and cytotrophoblasts invaded the wall of the blood vessels of the fallopian tube and sheets of trophoblasts and some villi were identified in the lumen of blood vessels. Multiple pulmonary nodules thought to be metastatic nodules were identified in the chest X-ray and serum beta-HCG had increased 2 weeks later. This case indicates that a careful pathological examination in the ectopic pregnancy is mandatory, because tubal GTD is not clinically distinguishable from ordinary tubal pregnancy.
Pregnancy ; Female ; Humans ; Neoplasm Metastasis

Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

OBJECTIVES: Estimation of the anterior lower uterine segment (LUS) thickness difference who underwent prior cesarean delivery measured with transvaginal sonography at or after 36 gestational weeks (sonographic thickness) and a ruler during elective cesarean section (operation thickness) Methods: One hundred sixty women who underwent prior cesarean delivery had the thickness of their LUS measured with transvaginal sonography at or after 36 gestational weeks. The LUS thickness was measured with a ruler during elective cesarean section. We compared group I whose LUS was fairly well visualized 4 cm or more from the uterine cervix to group II which had less than 4 cm. RESULTS: The mean sonographic thickness of LUS was 1.7 0.8 mm and that of operation thickness was 1.9 0.5 mm. The mean difference of the two (thickness difference) was 0.5 0.5 mm. In 31.3% the sonographic thickness was same as the operation thickness and in 70.7% of the total with 0.5 mm or less difference, the sonographic thickness could be regarded as accurate. The thickness difference with the sonographic thickness with 2 mm or more was smaller than those with 1 mm or less (0.4 0.5; 0.6 0.6) and that of group I was smaller than that of group II (0.4 0.4; 0.9 0.6) (p< .05). CONCLUSION: The thickness difference was 0.5 0.5 mm and it was smaller when the LUS thickness is 2 mm or over, clearly visible 4 cm or over from the cervix.
Cervix Uteri ; Cesarean Section ; Cicatrix* ; Female ; Humans ; Pregnancy ; Ultrasonography*

To investigate the effects of prostaglandin E1(PGX1) in prevention of proliferative scar formation, we cultured fibroblasts of normal skin (NS), hypertrophic scar (HS) and keloid (KL) tissues obtained from patients. We have compared type I collagenase production of cultured fibroblasts from normal skin, hypertrophic scar, and keloid tissues under various concentrations of PGE1. Our results demonstrate that type I collagenase production was significantly increased after addition of PGE1 in HS and KL, but not NS. Type I collagenase production of HS and KL fibroblasts were increased similarly in 10M and 10M of PGE1 and maximally increased in the concentration of 10M. This promotive effects of PGE1 on the production of type I collagenase was larger in KL than in HS. These results also suggest that PGE1 may play the promotive effects on type I collagenase production in dose-dependent manner. PGE1 may have a role in the prevention of hypertrophic scar and keloid by enhancing the production of type I collagenase of HS and KL fibroblasts. The promotive effects of PGE1 on type I collagenase production was variable depending on its concentration, and its effects was maximum in certain optimal condition. The maximally effective concentration of PGE1 in the prevention of proliferative scar formation should be searched in further investigations for clinical use.
Alprostadil ; Cicatrix ; Cicatrix, Hypertrophic* ; Collagenases* ; Fibroblasts* ; Humans ; Keloid* ; RNA, Messenger* ; Skin

Solar urticaria is a fairly uncommon but Well recognized clinical entity characterized by erythema and wheal with itching immediately following exposure to sunlight or artifical radiation. A 33-year-old female had a six-year duration of urticaria, tightness of chest and dizziness that appeared within 15 minutes of exposure to sunlight. The action spectrum of this patient was between 320 and 400nm and the passive and reverse passive transfer test were negative. Solar urticaria of our patient appeared to belong to type g in the classification of Harber et al(1963). The patient was treated with antihistamines and repeated exposure to sunlight for inducing tolerance. After 3 months of the treatment, the symptoms did not appear even after 3 hours of exposure to sunlight.
Adult ; Classification ; Dizziness ; Erythema ; Female ; Histamine Antagonists ; Humans ; Intradermal Tests ; Pruritus ; Sunlight ; Thorax ; Urticaria*

A case of scabies combined with superficial dermatophytosis which was aggravated by application of topical fluorinated steroid for 6 months is presented. One year ago a 60-year-old male began to have pruritic erythematous papules on the lower abdomen which spread slowly to the inguinal and buttock area. He had had onychomycosis for six years and for the last 6 months he had applied a. fluorinated steroid. preparation. After application of steroid, the aggravated skin lesions spread rapidly over the whole body. A KOH mount showed sarcoptes scabiei and fungus. A count of itch mites from scales brushed off and collected from. the overall surface of the patients body showed 263 adults, 169 Iarvae and 534 eggs. T. rubrum from a culture of scales from the trunk and nails was confirmed. On admission FBS and oral GTT test revealed diabetic curve, however it could not be determined whether the diabetes mellitus was primary or secondary to the use of topical steroid application. The patient has been treated with crotamiton cream and antifungal agent with remarkably effective results.
Abdomen ; Adult ; Buttocks ; Diabetes Mellitus ; Eggs ; Fungi ; Humans ; Male ; Middle Aged ; Mites ; Onychomycosis ; Ovum ; Sarcoptes scabiei ; Scabies* ; Skin ; Tinea* ; Weights and Measures

Painful fat hemiation is characterized. by the soft, skin colored painful papules on the heels that appears only when weight is bome and disappears when weight. is taken off the heels. The papules were thought to be caused by hemiation of adipose tissue through connective tissue defect. To our knowledge, such a case or similar one has not been reported previously in Korea. The patient was 30 year-old housewife, who have had soft, skin colored painful papules on the medial aspect of the both heels when weight was placed on the foot. Routine laboratory findings were within normal limit. Skin biopsy revealed nodules of lobulated adipose tissue on the mid-dermis far above the original subcutaneous tissue and large venous channels with thick muscular walls. Hypertrophied peripheral nerve trunks were scattered adjacent to the above vascular structure. Diagnosis was confirmed by clinical and histopathological features. Literatun was briefly reviewed.
Adipose Tissue ; Adult ; Biopsy ; Connective Tissue ; Diagnosis ; Foot ; Heel ; Humans ; Korea ; Peripheral Nerves ; Skin ; Subcutaneous Tissue

No abstract available.
Fetus* ; Humans ; Pregnancy, Twin* ; Twins*

No abstract available.
Fetus* ; Humans ; Pregnancy, Twin* ; Twins*