No abstract available.
Humans* ; Joints*

Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Humans ; Hypertrichosis ; Male ; Melanosis ; Nerve Fibers ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Pigmentation* ; Scoliosis ; Spine ; Young Adult

Supernunmerary nipple is a developmental anomaly occuring alon, the course of the embryological milk lines. This entity has receieved little attention in the dermatologic literature and has been confused with a pigmented nevus in some cases. We have experienced two ease of the more unusual form of supern umerary nipple. According to the Kajavas classification, our caes are classified as polithelia pilosa and complete breast with nipple.
Breast ; Classification ; Milk ; Nevus, Pigmented ; Nipples*

The reduction of nasal bone fracture has been done by a simple procedure. The nasal bone fracture was not a serious problem and patients are usually treated as outpatients. However it is causing increased demand for medical services far cosmetic reasons. We studied the nasal bone fracture by retrospective analysis, it comprising 606 patients with nasal bone fractures in various accidents and treated of Chonnam University Hospital from March 1995 to February 1998. We reviewed and analyzed the medical records and facial bone CT scans of 606 patients. The following results were obtained. 1. The prevalent age group was in the third decade, the most common cause was traffic accidents, while nasal swelling, tenderness, nasal deviation and crepitation were examined 2. The classification of nasal bone fracture was done by facial bone CT. Class 1: Nasal tip depressed fracture(16%) Class 2: Displaced nasal bone fracture without depression(30%) Class 3: Displaces nasal bone fracture with depression(12%) Class 4: Comminuted nasal bone fracture (10%) Class 5: Simple fracture of nasal bone and frontal process of maxilla(14%) Class 6: Comminuted fracture of nasal bone and frontal process of maxilla(11%) Class 7: Nasal bone fracture without displacement(9%) .
Accidents, Traffic ; Classification* ; Facial Bones ; Fractures, Comminuted ; Humans ; Jeollanam-do ; Medical Records ; Nasal Bone* ; Outpatients ; Retrospective Studies ; Tomography, X-Ray Computed

There is increasing evidence that inactivation of tumor-suppressor genes can promote tumor growth. Retinoblastoma protein (pRb) is the product of the retinoblastoma gene located on chromosome 13q14. pRb negatively regulates cell growth when functioning normally. Mutational inactivation of the Rb gene has been observed in retinoblastomas, osteosarcomas and soft tissue sarcomas. Recently, several other human cancers have also been shown to carry abnormalities of the Rb gene. The potential role of the Rb gene in cutaneous squamous cell carcinomas (SCCs) and basal cell caicinomas (BCCs), has not been determined and was the focus of this study. Immunohistochemical expression of pRb in 16 cutaneous SCCs and 17 BCCs was examined. The expression of PCNA was studied in parallel to assess the cellular proliferation rate in these lesions. The pRb and PCNA immunoreactivity were localized to the nuclei of tumor cells. A few pRb and PCNA positive cells were seen in normal squamous epithelium, sebaceous glands, sweat glands and hair follicles. The loss of expression of pRb was seen in 3 of 16 SCCs(18.8%) and 6 of 17 BCCs (35.3%). PCNA immunoreactivity was slightly high in pRb-negative or lower-positive cases. PCNA immunoreactivity was similar to that produced by pRb in some cases. These results suggest that mutational inactivation of the Rb gene may be related to the carcinogenesis of cutaneous SCC and BCC, though the frequency is relatively low.
Carcinogenesis ; Carcinoma, Basal Cell* ; Carcinoma, Squamous Cell* ; Cell Proliferation ; Epithelium ; Genes, Retinoblastoma ; Hair Follicle ; Humans ; Osteosarcoma ; Proliferating Cell Nuclear Antigen ; Retinoblastoma Protein* ; Retinoblastoma* ; Sarcoma ; Sebaceous Glands ; Sweat Glands

No abstract available.
Neurofibromatoses* ; Neurofibromatosis 1* ; Neurofibromin 1 ; Vitiligo*

No abstract available.
Humans ; Stenotrophomonas maltophilia*

Basal cell carcinoma(BCC) and squamous cell carcinoma (SCC) are very prevalent neoplasms of the human skin. Ultraviolet radiation in sunlight is a well-established mutagen of the p53 gene and is one of basal cell carcinoma and squamous cell carcinoma. The newly-formed vascular network is important for neoplasms to grow beyond a size of about 1 mm2. Recent reports have suggested the hypothesis that a mutant p53 protein is closely related with capillary density. Immumohistochemistry for p53 protein and CD34 was performed in 20 cases of BCCs and 14 SCCs to evaluated the relationship between p53 protein and capillary density. The results were as follows:1. The microvessels stained by CD34 were mainly located in the interface of tumor cells and stroma. 2. There was no difference in the microvessel density according to the histologic types and age of the patients, but a higher microvessel density was noted in male patients. 3. The aggressive BCCs and the less-differentiated SCCs showed higher p53 immunostaining. 4. The mean microvessel density of cases showing strong positive immunostaining of the p53 gene(54.73+/-17.75) was higher than that of others(39.75+/-18.30). These results suggested that p53 protein expression and microvessel density are not related to the histologic types and age of the patients, but that differentiation and biologic behavior such as the infiltrating property of tumors and the microvessel density are closely related to p53 protein expression.
Capillaries ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell* ; Genes, p53 ; Humans ; Male ; Microvessels* ; Skin* ; Sunlight

Talipes equinovarus deformity associated with arthrogryposis multiplex congenita(AMC) is rigid and its treatment is still controversial. The purpose of this study is to review the trend of recurrence after soft tissue release, and to delineate the risk factors affecting recurrence. Forty-one clubfeet in 24 AMC patients underwent soft tissue release as the first operation at the average age of 1 year 2 months (range, 6 months - 5 years 11 months) and were followed up for an average of 5 year 6 months (range, 2 years 1 month 14 years 5 months). Types of soft tissue release were Turcos posteromedial release (PMR) in 26 cases and posteromediolateral release (PMLR) using the modified Cincinati incision in 15 cases. Recurrences occurred in seventeen clubfeet (41%). A variety of recurrent deformity was corrected by redo PMR or PMLR, osteotomy, talectomy, arthrodesis, or Ilizarov technique. Average number of operations was 1.5 per case. Four parameters were analyzed in relation to recurrence: type of arthrogryposis, initial equinus deformity, type of primary soft tissue release, and age at the first operation. Recurrence occurred in proportion to the severity of initial equinus deformity, and was more frequent in amyoplasia type than in distal arthrogryposis type. However, neither the type of soft tissue release nor the age at the first operation was significant contributing factor to recurrence.
Arthrodesis ; Arthrogryposis* ; Clubfoot* ; Congenital Abnormalities ; Equinus Deformity ; Humans ; Ilizarov Technique ; Osteotomy ; Recurrence* ; Risk Factors

PURPOSE: Phyllodes tumor is a rare fibroepithelial disease of the breast that is classified as benign, borderline malignant, or malignant according to the pathological characteristics. To clarify diagnostic criteria for these groups, we analyzed the clinico-pathological characteristics of phyllodes tumors and the risk factors for local recurrence after surgery. METHODS: Between January 1997 and March 2003, we included 70 cases of recurring, pathologically-confirmed phyllodes tumors after surgery. Medical records were reviewed retrospectively for clinical characteristics and pathologic findings. RESULTS: The mean follow up period was 69.3 months (range 45.4 to 119.3 months) and mean age, 39.4 years old (range 18 to 63 years old). Benign phyllodes tumors were present in 45 cases (64.3%), borderline malignant tumors in 15 cases (21.4%), and malignant tumors in 10 cases (14.3%). A local recurrence developed in 9 cases (12.9%), with three of these cases benign (33.3%), 5 cases were borderline malignant (55.6%), and 1 case was malignant (11.1%). Borderline malignant cases showed a higher local recurrent rate (6.7%, 33.3%, and 10.0%, P=0.027). The local recurrence rate between the tumor-positive or close (<1 mm) resection margins and non-involved cases was different for benign (7.7% vs. 6.3%, P=0.031) and borderline malignant cases (60.0% vs. 20.0%, ns). CONCLUSION: Pathologic classification and small resection margins are risk factors for local recurrence in phyllodes tumors. Therefore, borderline malignant phyllodes tumors require wide excision with a sufficient resection margin or a mastectomy to prevent local recurrence.
Breast ; Follow-Up Studies ; Mastectomy ; Medical Records ; Phyllodes Tumor ; Recurrence ; Retrospective Studies ; Risk Factors