Dyskeratosis congsnita is a rare congenital disorder characterized by the triad of reticular pigmentation of the skin. dystrophic naila, and leukoplakia of the mucous membrane, and is often associated with severe pancytopenia. A 9-year-old boy had reticular pigmentation of the skin, dystropbic changes of the finger and toe nails, white patches of the buccal mucosa, mild hyperkeratosia of the palms and soles, excesaive lacrimation, dysphagia and severe pancytopenia, Bone marrow showed hypoplastic anemia and decreased cell mediated immunity was noticed.
Anemia, Aplastic ; Bone Marrow ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Deglutition Disorders ; Dyskeratosis Congenita* ; Fingers ; Humans ; Immunity, Cellular ; Leukoplakia ; Male ; Mouth Mucosa ; Mucous Membrane ; Pancytopenia ; Pigmentation ; Skin ; Toes

There have been no standard treatments for recurrent aphthous stomatitis, and clinical management is usually directed toward symptomatic relief. Recent immunological investigations have focused on possible imrnunopathogenesis of the disease. Several reparters suggested that levamisole, nonspecific immune-stimulator, had a beneficiaI effect in controlling attacks of recurrent a,phthous stomatitis and in reducing subsequent episodes. The present study was undertaken to evaluate the therapeutic effectiveness of levamisole in patients with recurrent aphthous stomatitis. A total of 8 patients who had had recurrent aphthous stomatitis for 2 to 20 years and who had experienced at least one episode per month were selected for this study from the department of dermatology, National Medical Center, through March 1979 to September 1979, Levamisole (Decaris') was given 150mg, p.o., once daily on 3 consecutive days every week for 2 months. Tbe results were as follows. 1) One patient had to have levamisole discontinued due to a high fever and exacerbations of tbe oral ulcerations. 2) 5 patients showed beneficial effects in reducing tbe number, frequency, pain and severity of recurrent aphthous stomatitis. 3') One patient showed no therapeutic response. 4) One patient, who has experienced new oral ulcerations continuously before starting levamisole, showed no recurrences of the lesions during the follow-up period of 3 months. 5) Side effects during levamisole administration were transient and generally mild, They included nausea, headache, dizziness and high fever.
Dermatology ; Dizziness ; Fever ; Follow-Up Studies ; Headache ; Humans ; Levamisole* ; Nausea ; Oral Ulcer ; Recurrence ; Stomatitis ; Stomatitis, Aphthous*

Epidermolysis bullosa aimplex is a dominantly inherited syndrome characterized by the dissolution of the basal epidermal cells with mechanical stress. A 23 year-old man developed recurrent bullous eruptions with pain on hands, feet and face since childhood, which healed spontaneously without scarring. Grandmother of his father's side, father, aunt of his father's side and two of his three brothers had suffered similar skin lesions. Light and electron microscopy showed degeneration and bulla formation in the basal cell layer of epidermis and intact basement membrane attached to dermis.
Basement Membrane ; Cicatrix ; Dermis ; Epidermis ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Fathers ; Foot ; Hand ; Humans ; Microscopy, Electron ; Siblings ; Skin ; Stress, Mechanical ; Young Adult

There are various complex formations between immunoglobulin and serum enzymes in the body. Of these, LD-Immunoglobulin complex is most common. It has reported that LD-IgG or LD-IgA complex existed in the serum of healthy person or many patients who have various clinical disorders. The clinical significance of complexes is not known, but it results in the increase of total LD activity and interference to the LD isoenzyme electrophoresis interpretation due to its anomalous pattern. We have reported a case of LD-Immunoglobulin complex in AILD(Angioimmunoblastic lymphadenopathy with dysproteinemia) with reference. The patient was admitted with fever, chills and cervical LN enlargement, in LN biopsy, diagnosed AILD, and during treatment expired by sepsis. Since admission, serum total LD activities were increased and serum LD isoenzyme EP showed that LD3-5 fractions was not separated in broad single band. Its pattern was due to LD-immunoglobulin complex, LD-bound immunoglobulin was IgG ,lamda. For the identification of immunoglobulin, we at first did serum protein immunoelectrophoresis. then stained the plate with tetrazolium dye for LD activity.
Biopsy ; Chills ; Electrophoresis ; Fever ; Humans ; Immunoblastic Lymphadenopathy* ; Immunoelectrophoresis ; Immunoglobulin G ; Immunoglobulins ; Lactic Acid* ; Lymphatic Diseases ; Sepsis

Acute febrile neutrophilic dermatosis, which was introduced to the dermatologic literature by Sweet in 1964, is characterized by persistent high fever and preceding upper respiratory infection like symptoms, polymorphonuclear leukocytosis in the peripheral blood, raised painful plaques on the limbs, face and neck, histologically a dense dermal infiltration with polymorphonuclear leukocyte, dramatic response to corticosteroids, and the absence of scarring. Case 1 was a 35 year-old female who had suffered from malaise, sore throat, and fever 3 to 7 days before the each episode of the cutaneous manifestation. Painful, red, raised, well-demarcated plaques appeared on the face with tendency of recurrences, and was accompanied by a few, small, shallow ulcerations on the lower Iip and tongue, and conjunctivitis. Polymorphonuclear leukocytosis (10400 with 65%, 11600 with 75%) and elevated ESR(23mm/hr, 19mm/hr) were found on two occasions. Histologically a dense inflammatory cell infiltration composed predominantly of polymorphonuclear neutrophils, small round cells, and a few eosinophils were seen in the dermis and subcutis, especially around the dermal capillaries. Alao there were many nuclear debris and marked endothelial cell proliferations. (countinued...)
Adrenal Cortex Hormones ; Adult ; Capillaries ; Cicatrix ; Conjunctivitis ; Dermis ; Endothelial Cells ; Eosinophils ; Extremities ; Female ; Fever ; Humans ; Leukocytosis ; Neck ; Neutrophils ; Pharyngitis ; Recurrence ; Sweet Syndrome* ; Tongue ; Ulcer

Malignant schwannoma is a rare spindle cell tumor derived from schwann cells. There is no significant age or sex distribution. The typical clinical presentation is that of a painless slowly enlarging soft tissue mass, occasionally associated with a peripheral neuropathy. Tumors tend to recur lacally and eventually metastasize, commonly to the lung. We report a case of solitary malignant schwannoma with metastsis to the regional axillary lymph nodes. The patient is a 50-year-old male who had the recurred tumor on right upper arm and child fist sized palpable painless mass on right axilla, which were confirmed to be malignant schwannoma and. lymph node metastasis. But we could not find any evidence of pulmonary metastasis. Tumor and metastatic lymph nodes were widely excised and skin graft was performed.
Arm ; Axilla ; Child ; Humans ; Lung ; Lymph Nodes ; Male ; Middle Aged ; Neoplasm Metastasis* ; Neurilemmoma* ; Peripheral Nervous System Diseases ; Schwann Cells ; Sex Distribution ; Skin ; Transplants

BACKGROUND: Hepatocellular carcinoma (HCC) is one of the leading causes of cancer deaths in the world, especially in Korea where 6-12% of the general population show positive for HbsAg. The accumulating studies suggest that the HBV-X protein found in HBV DNA may be the causative factor in the development of a HCC. METHOD: We studied the role of retinoblastoma (Rb) protein in the suppression of the tumorigenicity of a HCC by using cytomegalovirus (CMV) co-transfected HBV-X protein with retinoblastoma protein (pRb) or N-terminal truncated retinoblastoma protein (pRb94) in HepG2 cell lines. RESULTS: First, culturing HepG2 cells with CMV-Rb/liposome or CMV-Rb94/liposome, we observed the suppression of cell growth by using hemocytometric counting of the cells stained by trypan blue and by using a [3H]thymidine incorporation assay. Then, by using a plasmid co-transfected with the chloramphenicol acetyl transferase(CAT) gene, we investigated the role of HBV-X gene in regulating the transcriptional activity in the HepG2 cells under the control of a kB-like sequence of HIV-1 enhancer and the suppression of its activity by pRb and pRb94. CONCLUSIONS: We concluded that both pRb and pRb94 were capable of suppressing cell growth of a HepG2 cell line containing recombinant plasmids coding HBV-X protein. Furthermore, it was demonstrated that the suppression activity of pRb94 was more potent and sustaining than that of pRb. These results suggest that if additional research is performed on the method of gene delivery, gene therapy using pRb94 might be used as a new modality for the treatment of a HCC.
Carcinoma, Hepatocellular* ; Chloramphenicol ; Clinical Coding ; Cytomegalovirus ; DNA ; Genetic Therapy ; Hep G2 Cells ; Hepatitis B Surface Antigens ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; HIV Enhancer ; Humans* ; Korea ; Plasmids ; Retinoblastoma Protein* ; Retinoblastoma* ; Trypan Blue

No abstract available.
Acrodermatitis* ; Humans ; Siblings* ; Zinc Sulfate* ; Zinc*

The Parasitological investigation on the encysted metacercariae in brackish water fish, Tribolodon taczanowskii Steindachner , in the downstream of Hyungsan river which is located in Kyungpook province of Korea, were carried out, and the following results were obtained. Metacercariae of Metagonimus species were found in seventy five fishes (40.5 percent) out of 185 examined. Parasitic frequencies of the encysted matacercaria of Metagonimus species in Tribolodon taczanowskii were 23.2 percent (43 out of 185) in the scale, 7.0 percent (13 out of 185) in the gill, and 10.3 percent (19 out of 185) in the flesh. The number of the metacercaria in the scale, gill and flesh were fewer than that of the others. The worms were identified as Metagonimus yokogawai Katsurada, 1912 by morphological studies on the adults and eggs, and compared with prereported Metagonimus species.
parasitology-helminth-trematoda ; Metagonimus yokogawai ; epidemiology ; Tribolodon taczanowskii

Arcanobacterium haemolyticum, a aerobic Gram-positive rod, has been described as an unusual pathogen causing soft tissue infections such as pharyngotonsillitis, chronic ulcer and cellulitis. In addition, the microorganism causes deep-seated infection and systemic disease including endocarditis, vertebral osteomyelitis and sepsis in patients with predisposing conditions such as diabetes mellitus. Since colonies and microscopic findings of A. haemolyticum might be confused with those of streptococci and coryneform bacteria, and it is usually isolated with other microorganisms, it is often considered to be normal flora or a contaminant in wound infections, resulting in missed or delayed diagnosis. Streptococcus agalactiae infections in neonates and pregnant women have been well recognized. However, invasive S. agalactiae infections in non-pregnant older adults with chronic medical conditions, particularly diabetes mellitus, are increasing. We report a case of diabetic foot ulcer due to A. haemolyticum and S. agalactiae in an uncontrolled diabetes mellitus patient.
Adult ; Arcanobacterium ; Bacteria ; Cellulitis ; Delayed Diagnosis ; Diabetes Mellitus ; Diabetic Foot ; Endocarditis ; Female ; Humans ; Infant, Newborn ; Osteomyelitis ; Pregnant Women ; Sepsis ; Soft Tissue Infections ; Streptococcus ; Streptococcus agalactiae ; Ulcer ; Wound Infection