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

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

No abstract available.
Nasal Mucosa*

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.
Antibodies ; Antibodies, Monoclonal ; Biotin ; Bowen's Disease ; Coloring Agents ; Diagnosis ; Melanoma ; Molecular Weight ; Skin ; Streptavidin

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.
Actins ; Desmin ; Female ; Fibroblasts ; Follow-Up Studies ; Humans ; Infant, Newborn ; Myocytes, Smooth Muscle ; Myofibroblasts ; Myofibromatosis* ; Necrosis ; Parturition ; Pericytes ; Thorax

No abstract available.
Mycobacterium tuberculosis* ; Mycobacterium* ; Polymorphism, Restriction Fragment Length*

Optic nerve sheath meningioma arises from the arachnoid cap cell of optic nerve sheath and comprises most of primary orbital meningioma. We experienced a rare case of optic nerve sheath meningioma originating in the left orbit. A 44-year-old woman had suffered from visual disturbance in the left eye for 3 years and had only light perceptibility for the recent 6 months. The right eye was normal. Brain CT scan and MRI revealed a 2x0.8 cm sized fusiform solid mass in the left retrobulbar area. Under the impression of optic nerve tumor. excision of the mass including a small segment of the optic nerve was performed. The tumor was a yellowish firm, relatively well demarcated mass that encircled the optic nerve without infiltrating it. Microscopically it was a typical meningioma, meningothelial type. The recovery of visual acuity was impossible due to resection of optic nerve but there was no evidence of recurrence for u months.
Female ; Humans ; Meningioma

Allergic contact dermatitis due to 8-MOP is a rarely known si(ie effect of this widely used drug. Other known adverse reactions due to 8-MOP such as the oallergic dermatitis as well as some isolated cases of exanthema, papular eruptions, and astloma like symptoms are also sporadically reported. A 52-year-old man with vitiligo developed erythema to the UVA exposed 0.3% Oxoralen cream applied area. Prior to this episode, the patient had history of generalized burns after systernic PUVA therapy in 1983. Even after this experience, the patient had few more episodes of erythema at the site of 0.3%. Oxoralen cream application. We performed patch test and photopatch tests with Scandinavian series, 0.3% Oxoraler or am (as is), and diluted 8-MOP, 5-MOP, TMP solution. The result showed positive reactivity to 6-methylcoumarin, 8-MOP, as well as to 0.3% Oxoralen cream. The size of erythema was same in both irradiated areas which indicates an allergic contact dermatitis rather than photoallergic dermatitis or phototoxic dermatitis.
Burns ; Dermatitis ; Dermatitis, Allergic Contact* ; Dermatitis, Photoallergic ; Dermatitis, Phototoxic ; Erythema ; Exanthema ; Humans ; Methoxsalen ; Middle Aged ; Patch Tests ; PUVA Therapy ; Thymidine Monophosphate ; Vitiligo

BACKGROUND: The high level of susceptibility of patients with liver cirrhosis (LC) to septicemic infection is thought to be due to decreased reticuloendothelial function and irnpairment of several components of cell mediated and humoral immunity. It is well known that LC may cause many characteristic skin changes including jaundice, spider angioma, palmar erythema, xanthelasrna and nail changes such as clubbing and white nails. OBJECTIVE AND METHODS: During a 7 month period frorn April to October 1995, 100 patients who had been diagnosed as having LC were evaluated for the duration of their LC, the associated diseases they had apart from LC and various skin manifestations. Also, we performed the tuberculin test in 20 patients with LC and evaluated the correlation of the tuberculin test with severity of LC divided into 3 groups according to the Childs classification. RESULTS: The results are summarized as follows ; 1. The most frequent duration of LC was under 5 years (74%). 2. The most common associated disease of patients with LC was upper GI bleeding including esophageal varix (24 cases). Also, diabetes mellitus (13 cases) and hepatic coma (11 cases) were observed. 3. Jaundice (82 cases) was the most frequent skin rnanifestation of patients with LC. In addition, vascular changes (81 cases) and various nail changes (69 cases) were observed. Onychomychosis was the most common nail change of patients with LC. 4. 60% of the patients who were given the tuberculin test showed anergy, and the frequency of anergy increased in proport on to the severity of LC. However, it was not significant statistically (p> 0.05 ). CONCLUSIONS: Some of the skin manifestations help in the diagnosis of LC. Also, increased frequency of tuberculin test anergy in proportion to the severity of LC is thought to be related to decreased cell mediated immunity, although statistically it is not significant.
Child ; Classification ; Diabetes Mellitus ; Diagnosis ; Erythema ; Esophageal and Gastric Varices ; Hemangioma ; Hemorrhage ; Hepatic Encephalopathy ; Humans ; Immunity, Cellular ; Immunity, Humoral ; Jaundice ; Liver Cirrhosis* ; Liver* ; Skin Manifestations* ; Skin* ; Spiders ; Tuberculin Test