Several observation suggest that the antimelanocyte autoantibodies could play a role in melanocyte destruction. Some experiments indicate that melanocyte antibodies from patients with vitiligo can kill melanocyte in vitro. In these experiments, we demonstrated that vitiligo patient's sera containing antimelanocyte antibodies can lyse cultured human melanocytes by complement activation. Melanocyte cytotoxicity was measured using the ethidium bromide/ acridine orange viability assay. Significant melanocyte cytotoxicity was seen in sera from patients with both active and inactive vitiligo(p<0.01). Melanocyte cytotoxicity measured with complement-mediated cytotoxicity decreased after systemic steroid treatment(p<0.05) ; however melanocyte cytotoxicity showed no significant change with systemic PUVA therapy.
Acridine Orange ; Antibodies ; Autoantibodies* ; Complement Activation ; Ethidium ; Humans ; Melanocytes ; PUVA Therapy ; Vitiligo*

PURPOSE: Joubert syndrome presents neonatal respiratory abnormalities and other clinical manifestations. Pathologically the patients show hypoplasia or agenesis of cerebellar vermis and other intracranial anomalies. Our purpose is to evaluate the clinical manifestations and MR findings of Joubert syndrome. MATERIALS AND METHODS: Among the patients presenting with clinical stigmata of Joubert syndrome and agenesis of vermis on MR imaging, eight patients who did not satisfied the criteria of Dandy-Walker malformation, tectocerebellar dysraphia and rhombencephalosynapsis were selected. MR findings and clinical manifestation were analyzed. RESULTS: On MR imaging, agenesis of the cerebellar vermis (all cases), hypoplasia of the cerebellar peduncle (6cases), fourth ventricular contour deformity(6cases), tentorial elevation (4cases), deformity of the lateral ventricles (4cases), dysgenesis of the straight sinus (3cases) were demonstrated. Other findings were abnormalities of corpus callosum (3cases), falx anomalies (3 cases), occipital encephalomeningocele (2 cases) and fluid collection in posterior cranial fossa (2cases). Clinical manifestations were developmental delay (5cases), abnormal eyeball movement (3cases), hypotonia (2 cases), neonatal rerspiratoy abnormality (2cases), etc. CONCLUSION: Joubert syndrome showed various clinical manifestations and intracranial anomalies. MR imaging is an useful modality in detection of the cerebellar vermian agenesis and other anomalies of the patients.
Christianity ; Congenital Abnormalities ; Corpus Callosum ; Cranial Fossa, Posterior ; Dandy-Walker Syndrome ; Humans ; Lateral Ventricles ; Magnetic Resonance Imaging* ; Muscle Hypotonia

There are substantial evidences indiacting that anesthesia and surgery produce significant suppression of immune function. It is generally recognized that immunosuppressive manifestations such as lymphopenia, granulocytosis, decreased transformation to various antigens and impaired neutrophil chemotaxis could be followed by surgical operation. anesthetic agents may also have both direct and hormone-mediated indirect effects on immune systems. Recent evidences indicate that immunosurveillance mechanisms are not only important in the management of emerging infection and malignancy, but also critical to successful elimination of microscopic residual tumor after operation. This study was done to investigate the effeets of halothane anesthesia on T-lymphocyte subpopulations in patients undergoing hysterectomy. Lymphocyte, granulocytes and T-lymphoeyte subpopulations were counted before, during and after anestesia. The results showed that the decrease in the CD4+ cells (helper/inducer T-cells) was bigger than the decrease in the CD8+ cells (suppressor/ cytotoxic T-cells). CD3+ cells (total T-cells) and CD45R+ cells were also decreased. However, these decrease turned to normal values in 5 days. The overall result of this study suggest that anesthesia with halothane may bring temporal suppressive effect on the immunologic responses.
Anesthesia* ; Anesthetics ; Chemotaxis ; Granulocytes ; Halothane* ; Humans ; Hysterectomy ; Immune System ; Lymphocyte Subsets* ; Lymphocytes* ; Lymphopenia ; Monitoring, Immunologic ; Neoplasm, Residual ; Neutrophils ; Reference Values ; T-Lymphocytes

We investigated the clinical aspect and immunologic parameters of childhood and adult vitiligo in our clinics and made a comparative study. The childhood vitiligo showed the following results : similar incidence of clinical patterns with adults except for the lower incidence of the acrofacial type than in adults ; less involvement in the extent of lesion of disease than in adults ; higher incidence of family history than with adults ; of the precipitating factors, trauma is the main one whereas emotional stress is for adults ; higher association with halo nevi than adults ; and lower incidence of serum autoantibodies than adults. We have ascertained that childhood vitiligo is a distinct subset of vitiligo, showing the above features which will be studied in more patients in a long term follow-up clinical study.
Adult ; Autoantibodies ; Clinical Study ; Follow-Up Studies ; Humans ; Incidence ; Nevus, Halo ; Precipitating Factors ; Stress, Psychological ; Vitiligo

We report here a case of adenolipoma of the skin, an unusual variant of lipoma, arising on the neck. A 56-year-old man visited our hospital due to an anterior neck mass. An excisional biopsy was performed. The mass revealed a tan-yellow soft cut surface. We could not find any difference from other lipoma on gross inspection. Microscopically, the mass showed proliferation of mature adipocytes admixed with several eccrine units. The eccrine units were demonstrated by periodic acid-Schiff-positive granules in the secretory portions and by positivity of smooth muscle actin in the myoepithelial cells surrounding the eccrine glands. The tumor was completely excised, and the patient has been followed up without any evidence of recurrence so far.
Actins ; Adipocytes ; Biopsy ; Eccrine Glands ; Humans ; Lipoma ; Muscle, Smooth ; Neck ; Recurrence ; Skin

To estimate the child and infant mortality rates among Korean children, a mortality survey was carried out in the province of Kyongsangnam,buk-do. The study population are the beneficiaries of Korea Medical Insurance Coorporation (KMIC), Kyongsangnam,buk-do area, among which the 3,867 and 1767 deaths occurred from January, 1989 to December, 1990 in Kyongsangnam-do area and from January, 1991 to December, 1991 in Kyongsangbuk-do area respectively. We reviewed the data to confirm the causes of death. These were based on the death certificates and medical utilization records before dying which were available through the computerized databases on medical care utility of KMIC. The survey conducted along three steps. At first, the death certificates were examined, as a second step, medical utilization records were reviewed, and finally direct contacts to the family members of the deceased were done. As a result, 115 deaths were found under 20 years of age. Using them, age specific mortalities and causes of death were estimated. Mortality rate in this area was estimated 129 per 100,000 person-years during 1 month to 11 months of age, 67 during 1 year to 4 years of age, 23 during 5 years to 9 years of age, 10 during 10 years to 14 years of age, and 29 during 15 years to 19 years of age, respectively. And the frequent causes of death were congenital anomalies, central nervous system (CNS) diseases, certain condition originating from perinatal period during 1 months of age; accidents, congenital anomalies, malignancies, CNS diseases, and pneumonia or bronchiolitis during 1 to 4 years of age; accidents, malignancies, CNS diseases, and congenital anomalies during 5 to 14 years of age; and accidents, suicide, malignancies, and CNS diseases during 15 to 19 years of age.
Bronchiolitis ; Cause of Death ; Central Nervous System ; Central Nervous System Diseases ; Child ; Child Mortality* ; Child* ; Death Certificates ; Gyeongsangbuk-do ; Gyeongsangnam-do ; Humans ; Infant ; Infant Mortality ; Insurance ; Korea ; Mortality ; Pneumonia ; Suicide

Sunlight is one of the well-established factors which play key roles in the induction and exacerbation of lupus erythematosus. In two patients of discoid lupus erythematosus, we have experimentally reproduced skin lesions by provocative phototesting. Both UVA (100 joules/cm²) and UVB (80 millijoules/cm²) radiation induced the skin lesions. The reproduced skin lesions were clinically and histopathologically consistent with lupus erythematosus.
Humans ; Lupus Erythematosus, Discoid ; Reproduction* ; Skin ; Sunlight

BACKGROUND: PUVA has been used effectively in the treat,ment of vitiligo, but the mechanism by which PUVA stimulat.es melanocyte proliferation in vitiligo is not known. Several mechanisms have been suggested to be involved in the process of repigmentation of vitiligo. First, UV light, with or without psoralen, directly stimulates the proliferation of melanocytes. Secondly, PUVA may act. on epidermal keratinocytes or dermal components to stimulate t,hem to release certain melanocyte growth st,inulation factors that enhance the proliferation of melanocytes in depigmented lesions. Thirdly, PUVA irnmunologically leads to the impairment of epidermal Langerhans cell function and alteration of circulating T and B cell function, which results in the suppression of the stimuli is for rnelanocyte destruction during the therapy. OBJECTIVE: To test, th hypothesis that PUVA induced repigmentation in vitiligo results from the stimulation of growth factors that induce melanocyte proliferation, and that PUVA may suppress the immune reacticin to melanocytes, especially in autoantibody synt,hesis, we examined the effects of sera on the growth of epidermal melanocytes and control cells, and t,he incidence of antibodies to melanocyte and melanoma cells(SK-Mel 2~3) in the sera of patients with vitiligo. We also had normal control individuals and studied the changes of the antibody titer in the sera of patients with vitiligo. METHODS: The rate of H thymidine uptake was estimat,ed in cultured melanocytes and fibroblasts t,reated by patients sera before and after PUVA treatment. SDS-PAGE and immunoblotting analysis were used to idcntify anti pigment cell autoantibodies and were compared to the titers of autoantibodies after PUVA. RESULTS: 1. Melanocyte and fibrablast proliferation was increased by PUVA treated sera. Their proliferation was in proportion to the duration of the PUVA treatment. Melanocytes proliferated more than fibroblasts. 2. Significant differences between vitiligo patients and normal controls were found in the inci dence of anti-pigment cell antibodies. The antibodies were predominantly directed to melanocyte antigens of 110 kD, 65 kD, 45 kD and melanoma cell antigens of 110 kD, 103 kD, 88kD, 70 kD, 56 kD, 41 kD. 3. The titer of anti piment cell antibodies showed a tendency to decrease after PUVA treat- ment in most patients regardless of clinical improvement. Conclusion ; PUVA treated sera induced proliferation of melanocytes and fibroblasts and the production of aut,oantibodies was suppressed against pigment cell antigens through irnmunosuppression, which might help in the repigmentation of vitiligo.
Antibodies ; Autoantibodies* ; Electrophoresis, Polyacrylamide Gel ; Fibroblasts ; Ficusin ; Humans ; Immunoblotting ; Incidence ; Intercellular Signaling Peptides and Proteins ; Keratinocytes ; Melanocytes* ; Melanoma ; Thymidine ; Ultraviolet Rays ; Vitiligo*

BACKGROUND: PUVA has been used effectively in the treat,ment of vitiligo, but the mechanism by which PUVA stimulat.es melanocyte proliferation in vitiligo is not known. Several mechanisms have been suggested to be involved in the process of repigmentation of vitiligo. First, UV light, with or without psoralen, directly stimulates the proliferation of melanocytes. Secondly, PUVA may act. on epidermal keratinocytes or dermal components to stimulate t,hem to release certain melanocyte growth st,inulation factors that enhance the proliferation of melanocytes in depigmented lesions. Thirdly, PUVA irnmunologically leads to the impairment of epidermal Langerhans cell function and alteration of circulating T and B cell function, which results in the suppression of the stimuli is for rnelanocyte destruction during the therapy. OBJECTIVE: To test, th hypothesis that PUVA induced repigmentation in vitiligo results from the stimulation of growth factors that induce melanocyte proliferation, and that PUVA may suppress the immune reacticin to melanocytes, especially in autoantibody synt,hesis, we examined the effects of sera on the growth of epidermal melanocytes and control cells, and t,he incidence of antibodies to melanocyte and melanoma cells(SK-Mel 2~3) in the sera of patients with vitiligo. We also had normal control individuals and studied the changes of the antibody titer in the sera of patients with vitiligo. METHODS: The rate of H thymidine uptake was estimat,ed in cultured melanocytes and fibroblasts t,reated by patients sera before and after PUVA treatment. SDS-PAGE and immunoblotting analysis were used to idcntify anti pigment cell autoantibodies and were compared to the titers of autoantibodies after PUVA. RESULTS: 1. Melanocyte and fibrablast proliferation was increased by PUVA treated sera. Their proliferation was in proportion to the duration of the PUVA treatment. Melanocytes proliferated more than fibroblasts. 2. Significant differences between vitiligo patients and normal controls were found in the inci dence of anti-pigment cell antibodies. The antibodies were predominantly directed to melanocyte antigens of 110 kD, 65 kD, 45 kD and melanoma cell antigens of 110 kD, 103 kD, 88kD, 70 kD, 56 kD, 41 kD. 3. The titer of anti piment cell antibodies showed a tendency to decrease after PUVA treat- ment in most patients regardless of clinical improvement. Conclusion ; PUVA treated sera induced proliferation of melanocytes and fibroblasts and the production of aut,oantibodies was suppressed against pigment cell antigens through irnmunosuppression, which might help in the repigmentation of vitiligo.
Antibodies ; Autoantibodies* ; Electrophoresis, Polyacrylamide Gel ; Fibroblasts ; Ficusin ; Humans ; Immunoblotting ; Incidence ; Intercellular Signaling Peptides and Proteins ; Keratinocytes ; Melanocytes* ; Melanoma ; Thymidine ; Ultraviolet Rays ; Vitiligo*

Fibrous hamartoma of infancy (FHI) is an uncommon, benign, solitary intradermal or subcutaneous tumor. It occurs typically in the axillary or shoulder region. The histopathologic examination of affected lesion shows the characteristic elements: dense fibrous tissue, adipose tissue, and primitive mesenchymal tissues. A 15 month-old girl had the multiple, asymptomatic, discrete, and skin-colored tumors that scattered on the scalp. These were present at birth. We report a rare case of FHI occurred on the scalp with multiple and congenital characteristics.
Adipose Tissue ; Female ; Hamartoma* ; Humans ; Parturition ; Scalp ; Shoulder