We report a case of cutis verticis gyrata-mental deficiency syndrome associated with generalized epidermal nevus. The clinical features of the patient included severe mental retardation, drug resistant epilepsy, short stature, scaphocephaly and dysmorphic face with scanty scalp hair, hypertelorism, long palpebral fissure, low nasal bridge, hyperplasia of ala nasi, low-set ears with convoluted fold, and partial obstruction of the nasolacrimal ducts. Multiple convoluted folds and furrows on the scalp were extended to eyebrows and nose. Both lateral ventricle with periventricular leukomalacia were dilated markedly and the corpus callosum was hypoplastic on brain MRI. The nature of the yellowish brown colored elevated skin nevi on the whole body was consistent with systemic epidermal nevus by pathological study.
Brain ; Corpus Callosum ; Craniosynostoses ; Ear ; Epilepsy ; Eyebrows ; Hair ; Humans ; Hyperplasia ; Hypertelorism ; Infant, Newborn ; Intellectual Disability ; Lateral Ventricles ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging ; Nasolacrimal Duct ; Nevus* ; Nose ; Scalp ; Skin

We report a case of juvenile xanthogranuloma associated with neurofibromatosis. The patient was a 10-month-old female who had multiple yellowish papules on the face and shoulders A Biopsy of these lesions revealed foamy histiocytes with an admixture of inflammatory cells and Touton giant cells. Multiple, more than 6, cafe-au-lait spots, measuring more than 1.5cm in diameter were scattered on the trunk and buttocks Retardation of physical growth and development was observed.
Biopsy ; Buttocks ; Cafe-au-Lait Spots ; Female ; Giant Cells ; Growth and Development ; Histiocytes ; Humans ; Infant ; Neurofibromatoses* ; Shoulder ; Xanthogranuloma, Juvenile*

We report a case of juvenile xanthogranuloma associated with neurofibromatosis. The patient was a 10-month-old female who had multiple yellowish papules on the face and shoulders A Biopsy of these lesions revealed foamy histiocytes with an admixture of inflammatory cells and Touton giant cells. Multiple, more than 6, cafe-au-lait spots, measuring more than 1.5cm in diameter were scattered on the trunk and buttocks Retardation of physical growth and development was observed.
Biopsy ; Buttocks ; Cafe-au-Lait Spots ; Female ; Giant Cells ; Growth and Development ; Histiocytes ; Humans ; Infant ; Neurofibromatoses* ; Shoulder ; Xanthogranuloma, Juvenile*

Plexiform schwannoma is a relatively rare, benign peripheral nerve sheath tumor that can be located either in the deep soft tissues or in the dermis or subcutaneous tissue. This tumor may occur singly or as multiple lesions and may be localized to one anatomic site or diffusely distributed. Plexiform schwannoma should be differentiated with plexiform neurofibroma or other plexiform malignant tumors. We describe a case of a 6-year-old patient with multiple cutaneous plexiform schwannomas who had no other stigmata of neurofibromatosis 1 or family history suggesting a genetic disorder. The histopathological study revealed a tumor composed of multiple intradermal or subcutaneous interlacing and interconnecting fascicles and nodules that vary in size and shape. Characteristic Antoni A type cellular tissue showing frequent nuclear palisading and Verocay bodies were observed within well circumscribed elongated nodules.
Child ; Christianity ; Dermis ; Humans ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatosis 1 ; Peripheral Nerves ; Subcutaneous Tissue

This study was done for the evaluation of correlation among skin test reactivity, total serum IgE and RAST level in 202 atopic disease patients, who weie composed of 165 patients with allergic rhinitis, 22 patients with bronchial asthma, 11 patients with urticaria and 4 patients with atopic dermetitis. The results were as follows: 1. The sex ratio of male to female of 202 pat,ients with atopic disease was 1.3:1(114:88) and patients with age grciup of 2nd and 3rd decades occupied 64.9%. 2. The positive reactivity of the skin scratch test in general was 62.9%. The positive ratio was highest in 2nd and 3rd decades, 76.5% and 63.8% respectirely. The positive ratio was decreased as the age of patients increased. 3. The individual allergens which showed higher positive reaction in skin scratch test were D. farinae, house dust, cat hair, cockroach and Trichophyton ii descending order. In group allergens, the house dust groups consisted 45.5% of positiv. reaction of total number of the positive allergens. The positive ratio of aeroallergen was 84.5% whereas that of foods was only 11.5%. 4. The positive ratio of house dust group was 45.0% of total patients. 5. 41.1% of patients showed positive reactivity to two or more allegens while 21.8% was positive to single allergen. The most frequent combinations were 2 to 4 allergens(95.2%). 6. The mean of total serum IgE level in 63 patients who were poitiie skin scratch test was 470.5 IU/ml. 81.0% of these patients showed high serum IgE level. 7. As the positive reactivity of skin scratch test was increased, the mean level of total serum IgE was elevated(r=0.919). 8. A highly significant correlation was existed between the mear size of D. farinae scratch test reactions and the level of specific IgE to D. farinae(r=0.977).
Allergens ; Animals ; Asthma ; Cats ; Cockroaches ; Dust ; Female ; Hair ; Humans ; Immunoglobulin E* ; Male ; Rhinitis ; Sex Ratio ; Skin Tests ; Skin* ; Trichophyton ; Urticaria

This report concerns a rare malignant smooth muscle neoplasm of the skin and the subcutaneous tissue, which was examined immunohistochemically and ultrastructurally. It occured in a 48-year-old female patient who had suffered from painful nodules on the left shoulder. The nodules were removed and diagnosed as a benign fibrohistiocytic tumor. One year after local excision, multiple nodules were detected by physical examination at the previous operation site. Grossly, thirteen well-defined small nodules had spread in the dermis and the subcutaneous tissue, of which nine were located from lower dermis to the subcutaneous tissue and four were in the subcutaneous tissue. Histologically, each nodule consisted mainly of epithelioid tumor cells having eosinophilic or clear plump cytoplasm and round to oval nuclei with small nucleoli, and some peripheral spindle tumor cells. The tumor cells were negative for PAS reaction with and without diastase digestion or alcian blue. Immunohistochemically, tumor cells were positive for smooth muscle actin and vimentin. Ultrastructurally, the tumor cells showed actin filaments and dense bodies in cytoplasm. This case was diagnosed as an epithelioid leiomyosarcoma of the skin and the subcutaneous tissue with local metastasis one year after local excision.
Actin Cytoskeleton ; Actins ; Alcian Blue ; Amylases ; Cytoplasm ; Dermis ; Digestion ; Eosinophils ; Female ; Humans ; Leiomyosarcoma* ; Middle Aged ; Muscle, Smooth ; Neoplasm Metastasis ; Periodic Acid-Schiff Reaction ; Physical Examination ; Shoulder ; Skin* ; Subcutaneous Tissue ; Vimentin

The present study was carried out to investigate the HHsAg in the normal skin, dis- eased skin and skin specimens of window period of HBsAg positive patients in their serum with normal liver function. This study was done by immunoperoxidase techniques using the monoclonal antibody to hepatitis E3 surface antigen and by electron microscopy. Immunoperoxidase staining was dane on twenty specimeris of normal skin Group .D twenty specimens of diseased skin (15 cases of dermographism, 2 cases of purpura, 1 case of follicuht,is and 1 cases of morbilliform eruption) (Ciroup II ) and three specimens of normal skin af window period (Group III ) of HBsAp positive patients in their serum. Twelve cases of Group I, eleven cases of Group IL (1(J cases of dremographism and 1 rase of purpura) and two cases of Group II were stained positively for HBsAg. Positive sites were keratinocyte of epidermis, sweat gland, blood vessel and hair follicle. Electron microscopy failed to reveal viral particle, The above resuts suggest the possibility of transmission of viral hepatitis B thraugh the skin and skin appendages.
Antigens, Surface ; Blood Vessels ; Epidermis ; Hair Follicle ; Hepatitis B Surface Antigens ; Hepatitis B* ; Hepatitis* ; Humans ; Immunoenzyme Techniques ; Keratinocytes ; Liver ; Microscopy, Electron ; Purpura ; Skin* ; Sweat Glands ; Virion

We describe here a case of minimal deviation endometrioid adenocarcinoma (MDEA) of the uterine cervix that was initially suspected according to the abnormal cytologic findings in a 39-year-old woman. The Papanicolaou (pap) smear showed many neoplastic glandular cells in monolayered sheets, rosettes, and clusters with palisading and feathering borders. The tumor cells had oval, hyperchromatic nuclei, with chromatin clumping and small nucleoli. Histologic examination disclosed endometrial-type glands with a bland, isolated, mainly rounded appearance and these glands were widely scattered deep into the cervical stroma with only scant stromal reaction. An association of MDEA with tubo-endometrioid metaplasia or cervical endometriosis has been suggested by identifying the tubo-endometrioid glands in the vicinity of the MDEA.
Female ; Humans ; Adenocarcinoma

Primary tubal carcinoma is a rare carcinoma of the female genital tract, comparising with only 0.3% to 1% of female genital tract malignancies. Its histologic appearance and clinical behavior resemble that of primary ovarian carcinoma, thus the evaluation and treatment are also essentially the same. Presenting symptoms are variable, so pre-operative diagnosis is rare. A postmenopausal women presented with pelvic mass and vaginal discharge. It was initially diagnosed as ovarian tumor but primary carcinoma of the fallopian tube was confirmed postoperatively on the basis of the pathologic examination of resected specimen. So, we reported a case with a brief review of the concerned literature.
Diagnosis ; Fallopian Tubes* ; Female ; Humans ; Vaginal Discharge

BACKGROUND: The Korean Journal of Dermatology, a well-known dermatology journal in Korea, has been an es-sential medium for dermatologists and a symbol of the Korean Dermatological Association. The members of the associa-tion have an obligation to improve the quality of the journal. The statistical analysis is associated directly with the study design and completeness of an article. However, there has been no report on the statistical analyses used in the articles of the Korean Journal of Dermatology. OBJECTIVE: To investigate the usage of statistical methods in the journal and evaluate their accuracy and correctness. METHODS: A total of 264 original articles from 30 recent volumes of the Korean Journal of Dermatology (2003, vol.1 to 2005, vol.6) were collected and analyzed for statistical methods. The descriptive statistics were excluded from the study. The methods were con-sidered as 'correct', 'not-correct', or 'impossible to determine' in order to evaluate the correctness of the analyses. RESULTS: Of the 264 original articles, 126 (47.7%) articles included statistical analysis. The authors most frequently the used t-test and contingency table method. The incorrect analyses appeared in 30 (23.8%) articles, and their most frequently cited reason was their unwillingness to use the nonparametric analysis. CONCLUSION: Recently, the statistical methods in about one fourth of inferentially analyzed original articles of the Korean Journal of Dermatology have been found to be incorrect, and the reasons for the incorrectness appear not to be so difficult for authors to rectify.
Dermatology* ; Korea