Ehlers-Danlos syndrome is an inherited disorder of connective tissue in which joint hypermobility, hyperelasticity of skin, bleeding tendency, and scar formation are the most prominent features. It is a generalized disease with essentially universal involvement. A 3-year-old female child had velvety skin, skin hyperextensibility, joint hypermobility, subcutaneous mobile masses on the left shin, many scars on both knees, subcutaneous hematoma, and thick and very folded skin on both palms and soles, but no evidence of internal disorder. Cutaneous histopathologic findings were nonspecific without increase of dermal elastic fibers on Verhoeff stain. Diagnosis was confirmed by clinical and histopathological findings as Ehlers-DanIos syndrome.
Child ; Child, Preschool ; Cicatrix ; Connective Tissue ; Diagnosis ; Ehlers-Danlos Syndrome ; Elastic Tissue ; Female ; Hematoma ; Hemorrhage ; Humans ; Joint Instability ; Knee ; Skin

Report. about numeric change of LC between vitiliginous skin(VS) and adjacent normal appering skin (ANAS) are hard to find, Epidermal Langerhans Cell (LC) dersities in VS and ANAS were studied in 18 patient with generalized vitiligo. Adenosine triphosphatase(ATPase) stain was used to characterize LC. Epidermal LC densities were calculated by means of an eye piece reticule and expressed per mm. The results were as follows; 1. The was significant body site variation of LC densities in ANAS and the range of densities of LC per mm were from 668+165 to 1,241 _3 and the mean density of LC per mm was 944+258. 3. The LC densities of VS was similar to that of ANAS(p: not significant). In conclusion quantiative change of epidermal LC in vitiligo doesn't seem to have significant relation with pathogenesis of vitiligo.
Adenosine ; Humans ; Langerhans Cells ; Skin ; Vitiligo*

BACKGROUND: It is known that methylmercury poisoning, Minamata disease is very toxic to human body. But, cardiotoxic mechanism of methylmercury is left unknown, Recent study has been reported that the cleavage of methylmercury produce oxygen radicals as well as methyl radicals, and also these radicals induce the release of excitotoxic amino acids(EAAs). So, oxygen radicals and EAA are regarded as a causative factors in the various diseases such as heart disease induced by toxicity of methylmercury. We studied to know the cardiotoxic effect of methylmercury on cultured myocardial cells derived from neonatal rat in order to evaluate the toxic mechanism of methylmercury. METHODS: Myocardial cells of neonatal rat were incubated with various concentrations of methylmercuric chloride for 1-96 hours. MTT90 and MTT50 values were measured and cell viability was determined by MTT assay. In addition, morphological study was performed by light microscope after cultured myocardial cells that were exposed to methymercuric chloride. RESULTS: MTT90 and MTT50 values were 1microM and 15microM of methylmercuric chloride in cultured myocardial cells of neonatal rat respectively. Exposure of cultured rat myocardial cells to methylmercuric chloride resulted in a significant cell death in a time-dependent manner. In the observation of morphological changes, cultured cells treated with methlymercuric chloride showed decrease of cell number and disconnection between cultured myocardial cells. CONCLUSION: These observation suggest that methylmercury has a severe myocardiotoxicity on cultured myocardial cells derived from neonatal rat by the decrease of cell viability and morphological changes.
Animals ; Cell Count ; Cell Death ; Cell Survival ; Cells, Cultured ; Heart Diseases ; Human Body ; Mercury Poisoning, Nervous System ; Poisoning ; Rats* ; Reactive Oxygen Species

In healthy Korean adult males, 6 hour-MED was 51 2+22.6mJ/cm,24 hour-MED was 67. 5+26. 7mJ/cm, and MMD was 86. 5 t21. 3mJ/cm for UVB irradiation respectively. UVB-MED and UVB-MMD was increased by desoxymethasone, hydrocortisone, and bufexama.c creams. Hydrophilic ointment base increased only 24 hour-MED. For 1 MED of UVB, all test agents inhibited erytherna for 48 hours, For 2 MEDs of UVB, desoxymethasone, bufexamac; and hydrocortisone creams inhibited erythema at 6 hours, while desoxymethasone and hydrophilic ointment base could suppress erythema at: 24. hours after irradiation. However, desoxymethasone was the most effective. Hydrophilic ointment base was efficacious only at 24 hours after irradiation. For 1 MMD of UVB, desoxymethasone, hydrocortisone, and bufexamac creams could reduced the delayed tanning (DT) reaction.
Adult ; Anti-Inflammatory Agents* ; Bufexamac ; Desoximetasone ; Emollients* ; Erythema* ; Humans ; Hydrocortisone ; Male* ; Pigmentation* ; Tanning ; Triacetoneamine-N-Oxyl

Although the definition of male pseudohermaphroditism remains controversial, male pseudohermaphrodites will be defined as chromatin-negative individuals who have testis with the failure of normal development of the normal male. Also included are those individuals who may have more than one cell line, but at least one cell line, containing a Y chromosome and no cell line having two X chromosomes. Gonadal histologic findings may be either testicular or streak, but not ovarian. Male pseudohermaphroditism can result as a consequence of 1) absent M(llerian regression, 2) Inadequate synthesis of testosterone, 3) inadequate synthesis of dihydrotestosterone and 4) androgen receptor deficiency. We have seen 5 cases of male pseudohermaphroditism with manifestations of penoscrotal or perineal hypospadias, pubertal virilization, cryptorchism or atrophied testes, and feminization. Four cases had been reared as male and one case as female. All revealed chromatin-negative pattern in buccal smear and testes. After the plausible discussion, two cases were decided to be reared as female and three were as male.
46, XY Disorders of Sex Development* ; Androgen-Insensitivity Syndrome ; Cell Line ; Cryptorchidism ; Dihydrotestosterone ; Female ; Feminization ; Gonads ; Humans ; Hypospadias ; Male* ; Testis ; Testosterone ; Virilism ; X Chromosome ; Y Chromosome

Vitiligo is a relatively common depigmentary disorder occurring in approximately 1-2% of the general population. All races are affected. Both sexes are likely to be affected equally; the female prevalence in some studies can probably be attributed to cosmetic reasons. It can occur and spread at any stage of life and is often associated with a positive family history. Up to 30 percent of patients have reported vitiligo in another family member. The lesion is characterized by discrete, pale-white macules, few or several in number, which tend to enlarge centrifugally over time. It is not contagious, nor is it a serious health problem. However, it can be troublesome in brown and black people as well as in white persons who tan deeply (skin phototype IV), and often leads to social embarrassment and psychological turmoil.
Continental Population Groups ; Female ; Humans ; Prevalence ; Triacetoneamine-N-Oxyl ; Vitiligo*

BACKGROUND: Monofunctional psoralens plus UVA radiation are not severely phototoxic and have less mutagenic activity than bifunctional psoralens plus UVA radiation. OBJECTIVE: The purpose of this study was to evaluate pigment producing effect using various concentrations(0.02%, 0.1%, 0.5%) of monofunctional psoralens such as angelicin, khellin and comparing it's effect with TMP in topical photochemotherapy. METHOD: Ninty three C57BL mice were painted with either angelicin, khellin or TMP solution in concentrations of 0.02%, 0.1% and 0.5% each and were UVA irradiated. Skin biopsies were performed at 1,3,5 weeks after UVA irradiation. The pigment producing effects were measured by the number, area and perimeter of the melanocytes after topical PUVA. RESULTS: The comparison of melanocyte numbers between different psoralens after five weeks of photochemotherapy showed a significant difference in decreasing order of TMP, khellin and angelicin. The area and perimeter of melanocytes were larger in the TMP group after five weeks photochemotherapy than the other group. However in the khellin and angelicin group, the area and perimeter of melanocytes were not increased by increasing the frequency of the UVA irradiation. CONCLUSION: The number, area and perimeter of melanocytes after topical PUVA increased in the TMP group compared to angelicin or khellin group. We expect the clinical application of angelicin and khellin in vitiligo is possible considering the result of the study of pigment producing effect with a higher concentration and higher dose of UVA.
Animals ; Biopsy ; Ficusin* ; Furocoumarins ; Khellin ; Melanocytes* ; Methods ; Mice* ; Mice, Inbred C57BL ; Paint ; Photochemotherapy* ; Skin ; Thymidine Monophosphate ; Vitiligo

We herein report a 28-year-old woman with typical marginal keratoderma of the palms of 4 years duration. She denied any chronic pressure or trauma. Histological findings disclosed marked hyperkeratosis, parakeratosis, acanthosis, elongation of rete ridges, edema and capillary dilatations of papillary dermis, and infiltration of chronic inflammatory cells. There was no changes in collagenous and elastic fibers. Treatment with oral 13-cis retinoid and topical application of 0.02% flumethasone ointment containing 3%, salicylate resulted in only transit effect.
Adult ; Capillaries ; Collagen ; Dermis ; Dilatation ; Edema ; Elastic Tissue ; Female ; Flumethasone ; Humans ; Parakeratosis

A case of congenital self-healing histiocytosis was studied with S-100 antibody and electron microscopy. Many tumor cells were positive for S-100 protein and a few contained Birbeck´s grandles and dense bodies. A pathlogic fracture was first noticed on the seventh day. The lesions involuted spontaneously by the end of a weeks
Histiocytosis* ; Microscopy, Electron ; S100 Proteins

RePUVA therapy is the combination of an oral aromatic retinoid(etretinate) with oral photochemotherapy (PUVA) for the treatment of psoriasis. This combination therapy has the advantage of faster clearance with fewer side effects. Twenty three ambulatory psoriasis patients received RePUVA treatment. Etretinate was administered in a dosage of approxirnately 0.75 mg/kg body weight for 2 weeks and discontinued. One week after discontinuance of etretinate, PUVA therapy was begun. Complete clearance occured in 96% of the patients with an average of 9.5+4 irradiations and an accumulated ultraviolet A (UVA) dose of 67.5+42 J/cm. The primary side effects were dry rnucous membranes and mild cheilitis. Laboratory findings revealed no significant abnormalities before or after etretinate treatment.
Acitretin* ; Body Weight ; Cheilitis ; Etretinate* ; Humans ; Membranes ; Photochemotherapy* ; Psoriasis* ; PUVA Therapy