Intravascular fasciitis (IVF), a rare distinct variant of nodular fasciitis, possesses the same clinical and histologcal features of nodular fasciitis, but in addition, involves predominantly small to medium- sized veins and/or arteries intraluminally, intramurally and extramurally. Only 3 cases of intraoral IVF have been reported in the English literature. Herein we report a case of IVF developing on the inner lower lip and it was successfully treated with simple excision.
Arteries ; Fasciitis* ; Lip ; Veins

BACKGROUND: The hypereosinophilic syndrome(HES) represents a leukoproliferative process likely caused by a number of disorders, all of which are marked by sustained over-production of eosinophils. OBJECTIVES: The purposes of this study were aimed at evaluating the clinical and histopathological characteristics of HES. METHODS: The medical records and hist opathological slides of patients with HES who had skin biopsies performed in our department were reviewed. Criteria for the diagnosis of HES include (1) peripheral blood eosinophilia with eosinophil counts greater than 1,500/L for at least 6 months; (2) no evidence of parasitic, allergic, or other known causes of eosinophilia; and (3) presumptive signs and symptoms of multiple organ involvement. RESULTS: Four male and three female patients were included. HES developed in adulthood or old age (mean, 43.4 years). Because only the patients with cutaneous involvement were included, all the patients showed skin lesions. Heart, liver, stomach, nervous system, lymph nodes, and lung were involved organs in decreasing order of frequency. At the onset of HES, 40 to 80% of white blood cells were eosinophils in peripheral blood. White blood cell count and serum IgE level were elevated in all the tested patients. In six patients (85.7%), the level of serum erythrocyte sedimentation rate was elevated. Persistent hypereosinophilia (>1,500/L) was present for longer than 6 months in all patients. Stool examination and skin test for parasitic infestation all gave negative results. All the patients were not taking any medication. Histopathological examinations revealed perivascular mixed inflammatory cell infiltration; predominantly eosinophils in the stomach, liver, and nerve as well as in the skin. Interestingly, two patients who were presented with skin lesions showed the findings of eosinophilic vasculitis. In these patients, the skin lesions were consisted of Raynaud's phenomenon, digital gangrene, and several erythematous plaques. The most common cutaneous manifestations were papules and nodules on the extremities. The main treatment modality was systemic steroid. Except for one patient presented with central nervous system involvement of HES, all the patients were in a well-controlled state. In one patient with the typical clinical and hematologic features of HES, Hodgkin's disease followed. After the complete remission of Hodgkin's disease with chemotherapy, HES subsided. CONCLUSIONS: HES is a heterogenous collection of disorders marked by hypereosinophilia and organ damage. Most common cutaneous manifestations were papules and nodules on the extremities. Raynaud's phenomenon and digital gangrene can be the primary manifestation of HES in which cases cutaneous lesions showed eosinophilic vasculitis. Five patients (71%) responded well to systemic steroids. HES may be a herald of malignancy such as Hodgkin's disease. Further investigation will be mandatory ro elucidate the etiology and pathogenesis of HES.
Biopsy ; Blood Sedimentation ; Central Nervous System ; Diagnosis ; Drug Therapy ; Eosinophilia ; Eosinophils ; Extremities ; Female ; Gangrene ; Heart ; Hodgkin Disease ; Humans ; Hypereosinophilic Syndrome* ; Immunoglobulin E ; Leukocyte Count ; Leukocytes ; Liver ; Lung ; Lymph Nodes ; Male ; Medical Records ; Nervous System ; Skin ; Skin Tests ; Steroids ; Stomach ; Vasculitis

Laugier-Hunziker syndrone is a rare, pigmentary disorder of the lips, oral mucosa, and nails. The absence of intestinal polyposis differentiated it from Peutz-Jeghers syndrome. We describe a 16-year-old boy with multiple, brownish, lenticular macules on the lips, oral mucosa, perianal area and anal mucosa, and on the digits of his fingers and toes, in wham intestinal polyposis was not detectable.
Adolescent ; Fingers ; Humans ; Intestinal Polyposis ; Lip ; Male ; Mouth Mucosa ; Mucous Membrane ; Peutz-Jeghers Syndrome ; Toes

To understand better the complex natural course of HBV infection, the expression patterns of HBcAg and HBsAg in the liver of 51 inactive serum HBsAg carriers (24 CPH and 27 NPD) were studied by immunohistochemical methods. The inactive serum HBsAg carriers were devided into 3 groups by the following expression patterns of serum HBeAg/anti-HBe status and tissue HBcAg and HBsAg. Pattern A (18 cases) : HBeAg+, cHBcAg+ (94.4%), mHBsAg+ (61.1%), pATTERN B (14 cases) : anti-HBe+, nHBcAg+, cHBsAg+, Pattern C (19 cases) : anti-HBe+, HBcAg-, cHBsAg+ (89.5%). There were no significant differences between CPH and NPD, lthough the core free pattern was more common in the latter. The cHBcAg was expressed in 17 of 18 (94.4%) HBeAg seropositive cases but only one of 33 cases with serum anti-HBe, suggesting that the cHBcAg is intimately related to HBeAg. Since the inactive HBsAg carriers also expressed cHBcAg and/or mHBsAg, the necro-inflammatory activity of HBV infected liver is assumed to depend on the host immune response rather than their presence alone

BACKGROUND: Oculocutaneous albinism (OCA) is a genetic disorder of the melanin pigment system in which melanin synthesis is reduced or absent in the skin, hair, and eyes. OCA is classified into two major types, and tyrosinase-related OCA can be produced by mutations of the structural gene for tyrosinase enzyme (TYR gene). OBJECTIVE: The purpose of this study was to analyze the segregation of mutant alleles of the TYR gene in tyrosinase-negative and tyrosinase-positive Korean OCA patients and families. METHODS: We amplified exon I, II, and III of the TYR gene of Korean OCA patients and their families by polymerase chain reactions (PCR), and analyzed the mutations by restriction fragment length polymorphism (RFLP) analysis in exon I and single-strand conformation polymorphism (SSCP) analyses in exon II and exon III. RESULTS: Two tyrosinase-negative cases showed mutations in exon I. Four tyrosinase-nega-tive cases and one tyrosinase-positive case showed mutations in exon II, and one tyrosinase-neg- ative case showed mutations in exon III. In summary, we found three kinds of mutation in four tyrosinase-negative OCA patients and one tyrsinase-positive OCA patient. CONCLUSIONS: RFLP and SSCP analysis can provide a basis for a rapid and sensitive screening system to detect TYR gene mutations of Korean OCA patients and their families.
Albinism, Oculocutaneous* ; Alleles ; Exons ; Hair ; Humans ; Korea* ; Mass Screening ; Melanins ; Monophenol Monooxygenase ; Polymerase Chain Reaction ; Polymorphism, Restriction Fragment Length ; Polymorphism, Single-Stranded Conformational ; Skin

BACKGROUND: Previous studies have suggested that pityrosporurr yeasts, normal resident flora of skin, may be important in the pathogenesis of dandruff seborrheic dermatitis and confluent and reticulated papillomatosis. OBJECTIVE: We investigated the incidence and distribution of pityiosorum yeasts on clinically normal skin as a more reasonable method for providing the basic data or proving the relationship of pityrosporum and pathogenesi, of the diseases. MEHTODS: A total of 350 subjects were studied. Skin scrapings were stained with 20% Parker ink-KOH(P-KOH) solution. The numbers of pityrosporum spores and hyphe per high power field( x 400) were counted and designated according to a new grading method using bacterial index(BI) of lepra bacilli. RESULTS: The incidence rates of pityrosporum orbiculare(p. orbiculare) and pityrosporum ovale(p. ovale) were 60-70% and 20-50% of subjects respectively on seborrheic area., The incidence of p. orbicilare was highter than that of p. ovale on all examined sites except for the iterior scalp. And pityrosporal hyphae was present on anteriand posterior scalp and earwax in 24-25% of subjects respectively. The incidence of pityrosporum was higher in summer than other seasons The incidence rate of p. orbiculare and hyphae in the group aged below 9 (except neonates) and above 60, were lower than that of other age groups. The inciderice rates and distribution of grades of pityrosporum of neonates were lower than those of older subjects. CONCLUSION: The results of this study showed that the incidence and distribution of pityrosporum spores were similar to previoureports and hyphal forms were found 25% approximately on clinically normal skin of the scalp and carwax. The significance of the presenet of pityosporal hyphae and the relationship between normal skin and pityrosporal hyphae are to be firther elucidated.
Dandruff ; Dermatitis, Seborrheic ; Humans ; Hyphae ; Incidence* ; Infant, Newborn ; Malassezia ; Papilloma ; Scalp ; Seasons ; Skin* ; Spores ; Yeasts

BACKGROUND: Urticaria pigmentosa(UP) is primarily a disease of children. There have been no clinical studies of UP in Korea. OBJECTIVE: This study was performed to find the clinical characteristics of UP in Korean child-hood patients. METHOD: Twenty-nine cases of urticaria pigmentosa confirmed clinically and histopathologically were analyzed. RESULTS: The maculopapular type was the most common with the usual age of onset before the age of 6 months, and the lesions tended to be distributed in the central portion of the body. Darier sign was positive in 92% of the patients(24/26). No systemic involvements were detected in any of the patients with minimal associated symptoms. Seven patients of the maculopapular type and one patient of the multiple nodular type followed up for more than 2 years showed a tendency to improve or clear by the age of 6 years. CONCLUSION: Neonatal or infantile-onset patients of UP in Korean pediatric population were considered to have a benign clinical course and to require no aggressive therapy.
Age of Onset ; Child* ; Clinical Study* ; Humans ; Korea ; Methods ; Urticaria Pigmentosa* ; Urticaria*

No abstract available.
Diagnosis* ; DNA* ; Ichthyosis* ; Xeroderma Pigmentosum*

Quantitative analysis of the optic nerve head topography is important in diagnosis, differential diagnosis and follow-up of optic nerve diseases, especially in glaucoma. This study was undertaken to analyze the optic nerve head with confocal scanning laser ophthalmoscope(TopSSTM Software Version 2.2, LDT.Inc) and further to determine gender, age, refractive error and axial length-based on differences in the size and topography of the optic disc. One hundred normal human optic nerve heads of 100 subjects (42 men, 58 women, mean age 43.3+/-14.5 years) were evaluated. The men value of the parameters in TopSSTM were as follows : horizontal disc diameter 1.62+/-0.17mm, vertical disc diameter 1.77+/-0.24mm average disc diameter 1.71+/-0.15mm, disc area 2.31+/-0.43 mm2, cup area 1.01+/-0.50 mm2, horizontal cup to disc ratio 0.52+/-0.17, vertical cup to disc ratio 0.47+/-0.20, average cup to disc ratio 0.45+/-0.18, rim area 1.30+/-0.35 mm2. There were no significant correlations between these morphometric optic disc parameters and age, refractive error or axial length(p>0.05). However, male subjects had about 10% larger optic discs compared with female subjects. The optic disc size was well correlated with cup area and rim area (p<0.05).
Diagnosis ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Glaucoma ; Humans ; Male ; Optic Disk ; Optic Nerve Diseases ; Refractive Errors

BACKGROUND: Atopic dermatitis, characterized by dry, flasky, easily irritated skin, is the most common form of dermatitis seen during childhood. Infants with atopic dermatitis and other eczematous conditions need special dermatologic care to hydrate their skin and to minimize contact with irritants or allergens. OBJECTIVE: The purpose of our study is to evaluate the safety and efficacy of the skin products developed for children. METHODS: Twenty-eight normal children and 26 children with atopic dermatitis were evaluated clinically after using following commercal skin products : (lotion, cream, shampoo, soap, face wash wash) for 2 weeks. Water content of the skin of 14 volunteers was measured serialy over 8 haurs at 2 hour intervals after the application of each skin product. The skin pH of 16 volunteers was also measured serialy over 60 minutes at 30 minute intervals after application. We performed visual skin erythema grading, skin erythema grading by dermaspectrometer, transepidermal water loss measurement, skin water content measurement, and keratinocyte morphology observation by D-squame after the application of the skin products to 13 volunteers for 4 days.
Allergens ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Child ; Dermatitis ; Dermatitis, Atopic ; Erythema ; Humans ; Hydrogen-Ion Concentration ; Infant ; Irritants ; Keratinocytes ; Keratosis, Seborrheic ; Skin* ; Soaps ; Volunteers