Epidermolysis Bullosa Simplex is characterized by autosomal dominant rnheritance, and chronic noninflammatory conditions in which the clinical lesions, erosion, blisters usually result from relatively minor mechanical trauma to the skin, especially joints of hands, elbows, knees, and feet and other sites subject to repeated trauma The lesiona are present or appeared after a few days after birth or shortly after especialy of the lower legs or feet, and not involved at naiIs or mucous membranes. Though infection is not common on the feet or hands, even infected lesions generally heal with out scarring. 2 cases of Epidermolysis bullosa simplex were experienced, the one was 5 month old male baby who had fresh vesiculobullous, hemorrhagic bulla, crusts, exfoliation, no nail dystrophy, since a few days after birth. The another was 5 month old female baby, who had pea sized tense bulla, and exfoliations on the hands and feet. Diagnosis confirmed by clinical features and histological findings. Literature were reviewed in comparison with mechanobulIous disease.
Blister ; Cicatrix ; Diagnosis ; Elbow ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Female ; Foot ; Hand ; Humans ; Infant ; Joints ; Knee ; Leg ; Male ; Mucous Membrane ; Parturition ; Peas ; Skin ; Transcutaneous Electric Nerve Stimulation

Familial benign chronic pemphigus is characterized by a recurrent eruption of plaques of closely grouped vesicles that most frequently occurs about the neck, axilla and groin, singly or in combination with similar lesions in the intertriginous area. A 27-year-old male has had recurrent vesicles, fissures, maceration and crust formation in inguinal and perianal area for 3 yeare. The lesion manifested circinated form of vesicles, fissured and scaly patches over the inguinal, scrotal and perianal area. There were actively inflammatory border, resembling tinea cruris in the inguinal area. Authors diagnosed with clinical symptoms, laboratory examinations, and light and electron microscopic examination.
Adult ; Axilla ; Groin ; Humans ; Male ; Neck ; Pemphigus, Benign Familial* ; Tinea

PURPOSE: The neuophils contribute as an important defence mechanism against bacterial infection. In neonates with infection, not only the number of neutrophils are decreased due to inhibited production but also phagocytic function is impaired resulting in high mortality rate. The purpose of this study is to establish the effectiveness of exogenous granulocyte-macrophage colony-stimulating factor (GM-CSF) in infected neonates by comparing the serum levels of GM-CSF and various hematologic paratmeters in non-infected and infected neonates. METHODS: The study included 50 neonates without infection and 23 neonates with infection who were admitted to neonatal intensive care unit of Chungnam National University Hospital in between May 1998 and February 1999. The total number of white blood cell (WBC) counts, absolute granulocyte counts (AGC) and the serum GM-CSF concentration at birth were measured in non-infected neonates and they were stratified according to birth weight and gestational age. The total number of WBC counts, AGC and the serum GM-CSF concentration at postnatal 7th and 14th day were measured and compared with that of infected neonate's. Neonates with infection were divided either to receive exogenous GM-CSF & antibiotics or antibiotics only; The total number of WBC counts, AGC and the serum GM-CSF concentration at post-treatment 2nd and 7th day were measured and compared. RESULTS: Decreased total number of WBC counts, AGC and the serum GM-CSF concentration at birth were observed with lower birth weight and gestational age. In infected neonates, the total number of WBC counts, AGC and the serum GM-CSF concentration increased more when compared with that of non-infected neonates. Tendency towards increased total number of WBC counts, AGC and the serum GM-CSF concentration were observed after exogenous GM-CSF treatment. CONCLUSION: We recommend the use of GM-CSF in neonates with infection, especially those without increasing total WBC counts, and absolute granulocyte count as a norrnal physiological response.
Anti-Bacterial Agents ; Bacterial Infections ; Birth Weight ; Chungcheongnam-do ; Gestational Age ; Granulocyte-Macrophage Colony-Stimulating Factor ; Granulocytes* ; Humans ; Infant, Newborn ; Intensive Care, Neonatal ; Leukocytes ; Macrophages* ; Mortality ; Neutrophils ; Parturition

No abstract available.
Addison Disease*

No abstract available.
Humans ; Laparotomy* ; Nephrectomy* ; Tissue Donors*

Purpura fulminans (gangrenosa) is a rare clinical syndrome characterized by sudden appearance of rapidly progressive symmetrical subcutaneous ecchymosis durimg the convalescent stage of various infectious disease. A 4 year-old. boy has developed multiple skin defects with necrosis on extrernities and gangrenous changes on the left foot following a meningococcemia. B-K amputation on left leg and. free skin graft for multiple skin defect on extremities were, carried out.
Amputation ; Child, Preschool ; Communicable Diseases ; Ecchymosis ; Extremities ; Foot ; Humans ; Leg ; Male ; Necrosis ; Purpura Fulminans* ; Purpura* ; Skin ; Transplants

No abstract available.

No abstract available.
Child* ; Encephalitis, Arbovirus* ; Humans

Respiratory distress syndrome of preterm infants remains a significant cause of morbidyty and mortality. Early, just after birth, prediction and recognition of RDS is so important. The precision and reliability of the stable microbubble test (SMR)and shake test as a predictor of respiratory distress syndrome were studied. A 110-neonate who was born at Chungnam National University Hospital between November 1991 to September 1992was selected randomely and studied. The results were as follows; 1) Among the 110 neonates, 13 cases were noticed as RDS. 2) Among the 13 infants with RDS, SMR results were zero and very weak in 11 cases, weak in 2 cases. Of the 97 infants with Non-RDS, 9 cases were weak, 88cases were medium and strong, positive predictive value and negative predictive value was 100%, 98% respectively. 3) Of the 13 infants with RDS, Shake test result were negative in 8 cases, 1+in 1 case, 2+in 1 case and 4+in 3 case. Of the 97 infants with Non-RDS, 3 cases were negative, 9 cases were 1+, 9cases were 2+ and 63 cases were 4+, positive predictive value and negative predictive value was 72.7%, 9 However, frequent relapses and severe side effects caused by such therapy necessitate development of a more specific and effective therapeutic regimen.Recently, a T cell derived cytokine, interleukin 4 (IL-4)is being recognized as a major cytokine up-regulating IgE production and response, while interferon- (IFN- )counteracts IL-4 actions to down-regulate the IL-4 induced IgE response. Hence, the present study is aimed to investigate the role of IL-4 in MCNS. Using freshly isol 4.9% respectively. We conclude that the rapidity, simplicity and reliability of the stable microbubble test is more useful as a bedside procedure in identifying of predicting the infants who are likely to develop RDS than shake test.
Chungcheongnam-do ; Humans ; Immunoglobulin E ; Infant ; Infant, Newborn ; Infant, Premature ; Interleukin-4 ; Microbubbles* ; Mortality ; Parturition ; Recurrence

No abstract available.
Athletes* ; Female ; Humans