Paget's disease is classified, according to location, as mammary or extramammary disease. Extramammary Paget's disease especially in the vulva is extremely rare. Several hundred cases have been described worldwide. Patients with Paget's disease of the vulva are usually white and postmenopausal. The cause of which remains elusive. A case of vulva Paget's disease is presented with brief review of literature.
Humans ; Paget Disease, Extramammary ; Vulva

Porokeratosis may be due to an abnormal clone of cells, predisposing affected individusls to development of malignant neoplssm over involved area. A 58-year old female is prsented with basal cell epithelioma associated with PPPD, exhibiting the classical histopathologic criteria of the disease. Total excision for basal cell epithelioma and oral administration of etretinate for the treatment of porokeratosis plantaris, palmaris et disseminata and for the prevention of cancer developrnent resulted in good response.
Acitretin ; Administration, Oral ; Carcinoma, Basal Cell* ; Clone Cells ; Etretinate ; Female ; Humans ; Middle Aged ; Porokeratosis*

OBJECTIVE: The purpose of this paper is to study the possible differences in clinical and polysomnographic findings, depending on the presence or absence of subjective complaints of abnormal sleep behavior, in patients with RWA on polysomnography. METHOD: We reviewed patient records and polysomnographic data of patients referred to the Sleep Laboratory at Seoul National University Hospital from June 1996 through October 2002. We defined the manifest RBD group (n=32) as patients having both complaints of abnormal sleep behavior and RWA on polysomnography. The latent RBD group (n=20) consisted of patients who exhibited RWA on polysomnography but did not complain of abnormal sleep behavior. The clinical characteristics and polysomnographic findings between the two groups were compared and analyzed. RESULTS: Fifty-two subjects had RWA, as detected by polysomnography (42 males and 10 females, mean age of 55.1+/-19.1 years). Subjects in the manifest RBD group were significantly older than those in the latent RBD group (61.59+/-13.5 vs. 44.70+/-2.76 years, independent t-test, p<0.01). More subjects in the manifest RBD group exhibited abnormal REM behavior on polysomnography than did subjects in the latent RBD group (81.3 vs. 50.0%, Fisher's exact test, p<0.05). No significant differences between the groups were found in the prevalence of brain disorders and primary sleep disorders, gender proportion, and sleep architecture. CONCLUSION: No difference in sleep architecture was found between the manifest and the latent RBD groups. Only age and the presence of abnormal sleep behavior on polysomnography differentiated the two groups. We suggest that RWA on polysomnography without complaints of abnormal sleep behavior may be early manifestation of manifest RBD. Attention to RWA on polysomnography is necessary to help prevent full-blown RBD from developing.
Brain Diseases ; Female ; Humans ; Male ; Mental Disorders* ; Polysomnography ; Prevalence ; REM Sleep Behavior Disorder ; Seoul ; Sleep Wake Disorders ; Sleep, REM*

OBJECTIVE: The purpose of this study is to investigate the prevalence rate of OSA in subjects whose main sleep complaint is insomnia and to find differential factors of OSA in these insomniac subjects. METHOD: We reviewed the medical records and polysomnographic findings of patients referred to the Sleep Laboratory at Seoul National University Hospital from January 1996 to December 2002. Four-hundred and seventy subjects complained of insomnia as their main sleep problem (235 males and 235 females, mean age 53.6+/-12.4 years). First, we investigated the prevalence rate of OSA in these insomniac patients. Second, we compared the clinical and demographic characteristics of the OSA-associated group with those of the non-associated group. Third, we examined whether the degree or presence of differential factors within the OSA group correlate with severity of OSA, as determined by the respiratory disturbance index (RDI). RESULTS: Among 470 insomniac subjects, 125 subjects (26.6%) were diagnosed as OSA by nocturnal polysomnography. OSA-associated subjects were significantly older (58.4+/-12.3 years vs. 51.8+/-11.2 years, p<0.01), and had significantly higher body mass index (BMI) (23.4+/-3.3 kg/m2 vs. 22.5+/-3.1 kg/m2, p=0.44) than non-associated subjects. The OSA-associated group had more subjects with male gender (64.0 % vs. 44.9 %, p<0.01), hypertension (20.0 % vs. 9.3 %, p<0.01) or snoring (96.0 % vs. 63.5 %, p<0.01). Within the OSA-associated group, age had a significant positive correlation with RDI (p=0.01). CONCLUSION: We found that a considerable portion of patients complaining of insomnia as their main sleep problem were diagnosed as OSA. Snoring, old age, male gender, obesity, and comorbid hypertension were found to be differential factors of OSA in insomniac patients. We suggest that diagnostic efforts including nocturnal polysomnography are needed for insomniac patients with any of the above risk factors of OSA.
Body Mass Index ; Female ; Humans ; Hypertension ; Male ; Medical Records ; Obesity ; Polysomnography ; Prevalence ; Risk Factors ; Seoul ; Sleep Apnea, Obstructive* ; Sleep Initiation and Maintenance Disorders* ; Snoring

A 38-year-old woman was presented with a dark brown plaque on the abdomen. Clinically, the tumor was simulating the appearance of dysplastic nevus. Microscopically, the cells of the stratum malphighii lay in a disordered pattern. Many cells in the epidermis were atypical and melanin pigment was mainly in the basal layer of the epidermis and the upper dermis. Diagnosis of pigmented Bowen's disease was made. Pigmented Bowen's disease is rarely found at body sites other than the anogenital area.
Abdomen ; Adult ; Bowen's Disease* ; Dermis ; Diagnosis ; Dysplastic Nevus Syndrome ; Epidermis ; Female ; Humans ; Melanins

Apocrine poroma is a benign cutaneous adnexal neoplasm differentiating in the direction of sebaceous and apocrine glands, and follicular germs. The clinical appearance of apocrine poroma is not distinctive, and the histologic finding is similar to that of eccrine poroma, which is typified by proliferation of poroid and luminal cells in continuity with the epidermis. But sebaceous, apocrine or follicular differentiation may also be found in the case of apocrine poroma. We herein report a case of apocrine poroma on the scalp. This case exhibited apocrine and sebaceous differentiation, and connection to an adjacent follicular epithelium.
Apocrine Glands ; Epidermis ; Epithelium ; Phenobarbital ; Poroma* ; Scalp

Mixed tumor of the skin (chondroid syringoma) is a rare benign tumor composed of epithelial elements intermingled with myxoid or cartilagenous stroma which is not separated by basement membrane. It had been believed to originate from the eccrine gland but recently, it was described to be of apocrine gland origin in case of showing apparent apocrine secretion. We report on a 63-year-old man with a tumor on the right upper eyelid showing typical microscopic features of mixed tumor of the skin. Many apocrine decapitation secretions were seen in tubular structures and follicular differentiations were also seen, which represents the common origin of folliculo-sebaceous-apocrine unit.
Adenoma, Pleomorphic ; Apocrine Glands ; Basement Membrane ; Decapitation ; Eccrine Glands ; Eyelids ; Humans ; Middle Aged ; Skin

Blue nevus is a benign melanocytic neoplasm and represents itself usually as a solitary blue or blue-black papule. It rarely occurs as multiple lesions grouped in a circumscribed area. How-ever, non-grouped disseminated blue nevi are exceedingly rare. We report a patient with acquired multiple blue nevi that was distributed over the entire body discretely and showed an increase in the number of the nevi without any causal factors.
Humans ; Nevus ; Nevus, Blue*

OBJECTIVES: The purpose of this study was to assess the clinical and polysomnographic characteristics of Korean patients with obstructive sleep apnea syndrome (OSAS), especially in relation to differences due to age and gender. METHODS: All subjects were consecutive patients who were proven to have OSAS with nocturnal polysomnography. They were interviewed with a structured interview format including sociodemographic information, past medical history, medication, and sleep-related history. Simultaneously, they were also given Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS) to answer in order to check subjective sleep quality and subjective sleepiness. RESULTS: Mean age of the 308 subjects was 49.5+/-13.3 years, with 77.6% of the subjects being males and 22.4% of the subjects being females. The aging effects on the sleep architecture in Korean OSAS corresponded with normal aging, but with the effect of OSAS itself superimposed, the extent of aging effects was more marked than that of normal aging. The severity of Korean patients of OSAS was not correlated with age. When divided into age subgroups, significant correlation was found between RDI and BMI in patients of each subgroup of those in the 4th to 7th decades. The oldest subgroup (>70 years) described their subjective sleep quality as poorer than any other age subgroups, despite of less subjective drowsiness. The severity of OSAS and the change of sleep architecture of male subjects turned out to be severer than those of female ones. The female/male ratio of the subjects tended to increase with aging. CONCLUSIONS: The aging effect on the sleep architecture in Korean OSAS seems to be a mixture of the changes by normal aging and sleep disorder per se. The severity of OSAS was not correlated with age, but highly correlated with BMI. The severity of OSAS and the change of sleep architecture of male patients were severer than those of female ones.
Aging ; Body Mass Index ; Female ; Humans ; Male ; Polysomnography ; Sleep Apnea, Obstructive ; Sleep Stages

Plexiform neurofibroma is considered to be pathognomic of neurofibromatosis type 1 (NF1). Herein we report a solitary plexiform neurofibroma which is not associated with NF1. A 61-year-old man presented with asymptomatic skin colored nodules on the medial side of his left great toe. No other abnormalities were found in his personal or family history. Clinically, the tumor was simulating the appearance of mucous cysts. Microscopically,it was a plexiform neurofibroma located in the dermis which seemed to originate from small superficial nerves. This case would seem to confirm that the superficial form of plexiform neurofibroma involving small nerves in the dermis or subcutis is not necessarily pathognomic for NF1.
Dermis ; Humans ; Middle Aged ; Neurofibroma, Plexiform* ; Neurofibromatosis 1 ; Skin ; Toes