The blue rubber bleb nevus syndrome(CRBNS) is a rare disorder characterized by distinctive vascular malformations of the skin and gastrointestinal tract. The syndrome is associated commonly with iron deficiency anemia due to gastrointestinal bleeding. Most cases are sporadic, although a few have been reported with autosomal dominant inheritance. We report a case with clinical characteristics of the blue rubber bleb nevus syndrome. The patient had typical skin manifestations of the BRBNS and chronic iron deficiency anemia. However, we could not find any gastrointestinal vascular malformations radiologically.
Anemia, Iron-Deficiency ; Blister* ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Nevus* ; Rubber* ; Skin ; Skin Manifestations ; Vascular Malformations ; Wills

No abstract available.
Cystic Adenomatoid Malformation of Lung, Congenital* ; Lung*

BACKGROUND: Pilomatricoma(Benign calcifying epithelioma of Malherbe) is a uncommon benign tumor with differentiation toward hair cortex cells. It commonly manifests itself as a firm, deep seated nodule with normal Overlying skin, However, anetodermic cutaneous changes have also been described in the literature since 1943. Although the authors hypothesized this phenomenon as a chronic mechanical irritation or a step of transepidermal elimination of the tumoral mass, distinctive elucidation of pathomechanism of it remained. OBJECTIVE: The purpose of this study was to characterize the clinical and histopathologic features of 25 cases with pilomatricoma and to elucidate pathomechanism of the anetodermic cutaneous changes through the comparison of the anetodermic pattern of overlying skin with the changes of the tumoral mass or collagen and elastic fibers in the dermis. METHODS: Twenty-five cases with pilomatricoa have been collected at Chunganam National University Hospital and Kongju Medical Center during 15 years and 5 years, respectively, And we studied the clinical and histopathological characteristics of patients and tried to reveal the relationship between the cutaneous manifestations and the histologic changes through the test for independence. RESULTS: 1. In the age distribution, the patients lesser than 30 years old was 23 cases(92%). The mean age was 18.9 years old. The ratio of males to females was 1 : 2.6. 2. The duration of lesions was from 2 months to 7 years, with the mean duration of 11 months. The predilection sites were arm(40%), face(28%), neck(12%), shoulder(8%), scalp(4%) and back(4%) in order. All lesions were hard in consistency, and were from bean to walnut sized. Clinically, 10 cases (40%) showed reddish discoloration and/or anetodermic cutaneous changes. 3. Histopathologically, the typical patterns of pilomatricoma were commonly located at the dermis (23 cases) and consisted of the shadow cells predominantly(21 cases). Calcium deposits(21 cases, 84%) were noted within the shadow cells. Eight cases(32%) had the tumoral maese demarcated with fibrous capsules, and the foreign body reaction with giant cells and chronic mononuclear cell infiltrations around the shadow cells ruptured the fibrous capsules in 17 cases(68%). 4. When we compared the relationship between the inflammatory cell infiltrations around the tumoral mass and the changes of dermal collagen and elastic fibers, we observed that the changes of dermal fibers and foreign body reaction and mononuolear cell infiltrations were statistically co-related. When compared the cutaneous changes with the inflammatory cell infiltrations of the tumoral masses, we could also reveal that the anetodermic cutaneous changes of the overlying skin might be influenced by the foreign body reactions with chronic mononulcer cell. Based on the status of foreing body reaction, the average of onset was inversely proportional to the average of age. CONCLUSION: Pilomatricomas are slowly growing, firm, deep seated benign tumor which occurs commonly on the arm, face and neck. However, they often showed the anetodermic changes on the overlying skin. Anetodermic cutaneous changes or discoloration may be correlated with the degree of foreign body reactions without the evidences of transepidermal elimination histopathologically.
Adult ; Age Distribution ; Arm ; Calcium ; Capsules ; Carcinoma ; Chungcheongnam-do ; Collagen ; Dermis ; Elastic Tissue ; Female ; Foreign Bodies ; Foreign-Body Reaction ; Giant Cells ; Hair ; Humans ; Juglans ; Male ; Neck ; Pilomatrixoma* ; Skin

No abstract available.
Rhinoplasty*

BACKGROUND: The skin changes of X-linked recessive ichthyosis are cnused by the deficiency of the enzyme steroid sulfatase, which usually results from deletions of this gene in Caucasian populations. OBJECTIVE AND MEHTODS: To disgnose X-linked recessive ichthyosis and detect its carrier, we have investigated distinctive gene deletion and measured gene dosage of steroid sulfatase gene by southern blot hybridization in Korean patients with X-linked recessive ichthyosis. RESULTS: Patients from 8 of 9 unrelated families exhibited deletions, if the steroid sulfatase gene. Of 6 families showing a family history compatible with X-linked recessive inheritance, One family exhibited a normal pattern of hybridization. All but one family showed deletion of steroid sulfatase gene. All three patients lacking a fami1y history of the disease exhibited gene deletions. The ratio of the steroid sulfatsse specific band density to the Factor VIII specific band density was measured in 8 obligate carriers using a laser densitometer. The average ratio exhibited by the car riers was less than half that of normal women. Conclusian: These results suggest that the X-linked recessive ichth osis patient and its carrier can also be diagnosed and detected by Southern blot hybridization of steroid sulfatase gene in Korea.
Blotting, Southern* ; Diagnosis* ; Factor VIII ; Female ; Gene Deletion ; Gene Dosage ; Humans ; Ichthyosis* ; Korea ; Skin ; Steryl-Sulfatase ; Wills

The 1,783 patients with eye injuries, including the 234 in-patients, who visited the Department of Ophthalmology, Seoul Adventist Hospital from Jan. 1, 1987 to Dec. 31, 1988 were clinically analyzed. The results were as follows: 1. The incidence of ocular injuries was 4.7% of all eye patients, and 25.5% of all patients admitted to this ophthalmologic department. 2. The incidence was more common in male(76,8%)and in the age of 3rd to 4th decades(47.0%). 3. The ocular injuries were more common in the spring(27.3%), and in March. 4. The most common cause of ocular injuries was sand or dust or iron piece(29.8%), followed by fist or finger(12.2%) and traffic accident(12.5%), but the injuries by iron products(20.1%) were the most common in the admitted patients. 5. The most common ocular injury was conjunctival foreign body(23.7%), followed by subconjunctival hemorrhage (20.6%) and (9.5%). In the cases of in-patients, hyphema(24.2%) was the most coommon, followed by corneal lacera tion(12.2%) and eyelid laceration(11.2%). 6. Surgical procedure included corneal suture(18.3%), primary closure of eyelid and face(22.2%). 7. Visual acuity was improved in most cases by treatment, but the corrected vision after treatment was less than 0.1 in 20.5%, which was mainly due to the perforating eye injuries. 8. The most common complication of ocular injuries after treatment was corneal opacity(34.1%), followed by vitreous opacity(9.4%).
Dust ; Eye Injuries ; Eyelids ; Hemorrhage ; Humans ; Hyphema ; Incidence ; Iron ; Ophthalmology ; Seoul ; Silicon Dioxide ; Visual Acuity

PURPOSE: To describe the high-resolution CT findings of varicella-zoster pneumonia. MATERIALS AND METHODS: Seven adult patients with clinically and serologically diagnosed varicella-zoster pneumonia underwent HRCT scanning. One had undergone a kidney transplant, and the others were immunocompetent. The HRCT findings were retrospectively analyzed by two radiologists in terms of the presence of a nodule, pneumonic consolidation, ground-glass attenuation and pleural effusion, and with regard to the number, size and distribution of nodules. RESULTS: HRCT findings of varicella pneumonia included nodule (n=7), consolidation (n=3), diffuse groundglass attenuation (n=1) and pleural effusion (n=1). Nodules were mostly 2-10 mm in size, and multiple (n=6), and zonal predominancy was not apparent. One case in which HRCT demonstrated diffuse groundglass opacity and slight bilateral pleural effusion involved a patient in whom complications of acute respiratory distress syndrome occured. CONCLUSION: When HRCT demonstrates the presence of nodules 2-10 mm in size multiple nodules, or nodule surrounded by ground-glass attenuation, or the coalescence of nodules and consolidation is observed in adults with chickenpox, the varicella-zoster pneumonia should be included in the differential diagnosis.
Adult ; Chickenpox ; Diagnosis, Differential ; Fluconazole ; Humans ; Kidney ; Pleural Effusion ; Pneumonia* ; Respiratory Distress Syndrome, Adult ; Retrospective Studies

Bullae and sweat gland necrosis have been often described in patients with mental ges, whieh commonly alanifested as erythematous or vesicobullaus lesions on pressure sites. Histopathologically, the aecrosis of sweat glands is a characteristic 6nding. Generalized and/or loca1 tlssue bypoxia due to prolonged ieamobihxation may have resulted in these clinical and histopakological manifestations. To date, we have experielwed 15 cases with bulla and sweat gland necrosis. On admission, about half the patients had carbon monoxide poisoning, and the remainders had alcohol overdosage, drug inioxication, and others. A11 our patients had menfal changes caused by carbon wonoxide poisening, aleohol intoxication, and others. Abrupt erythematous swelling and/or vesicobullous lesione affected pressure sites in all patients. Three patietns had erythematoias lesiqms on non-pressure sites simuhtaneously. Histopathologic examinatians of 12 biopsy specimes showed the necrosis of the epidermis, intra- or sub-epidermal bulla, and/or sweat gland necrosis.
Biopsy ; Carbon ; Carbon Monoxide Poisoning ; Epidermis ; Humans ; Necrosis* ; Sweat Glands* ; Sweat*

We report a case of rosacea in a 65 year old female with a 14 year history of polycythemia vera. The patient suffered from several constitutional symptoms and signs suggestive of polycythemia vera. Six years prior to our initial examination, erythematous lesions were first noted on the center of the face. These lesions exhibited periodic improvement and exacerbation without specific treatment. Histopathologic examination of the facial lesions showed nodular infiltration of lymphocytes and histiocytes and dilation of blood vessels. On the basis of laboratory examination, the patient was diagnosed as polycythemia vera. The patient has received metronidazole, tetracycline, and topical steroids to control rosacea-like facial lesions. The treatment results were not significant. However, phlebotomy markedly improved the skin lesion. To our knowledge, there are no reports describing the clinical course of rosacea lesions following the treatment with phlebotomy.
Blood Vessels ; Female ; Histiocytes ; Humans ; Lymphocytes ; Metronidazole ; Phlebotomy ; Polycythemia Vera* ; Polycythemia* ; Rosacea* ; Skin ; Steroids ; Tetracycline

To understand the role of Transglutaminase K(TGase K) in skin diseases, the expression of TGase K protein was studied in hyperkeratotic inflammatory skin diseases, and in several different kinds of benign and malignant skin tumors. Immunohistochemistry was used to detect the TGase K protein with a new anti-human TGase K polyclonal antibody. This antibody showed the same immuno- histochemical staining pattern as in previously published immunofluorescence data with the exception that the immunohistochemical stain showed a slightly weaker stain in the granular layer. However, the staining intensity was decreased in psoriasis, which is opposite for results obtained with the widely used TGase K monoclonal antibody B.C1. In other hyperkeratotic inflammatory skin diseases, like pityriasis rubra pilaria, lichen planus, and pityriasis rosea, the staining patterns were similar while the staining intensity was decreased, compared to normal epidermis. Squamous cell carcinoma showed a diffuse staining pattern in tumor cells with a small intensity increase in well differentiated tumor cells. However, in actinic keratosis and Bowen's disease, which are thought to be precursors of squamous cell carcinoma, the staining intensity was weak. The tumor cells of basal cell carcinoma, malignant melanoma, and seborrheic keratosis showed a weak staining intensity.
Bowen's Disease ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Epidermis ; External Fixators ; Fluorescent Antibody Technique ; Immunohistochemistry ; Keratosis, Actinic ; Keratosis, Seborrheic ; Lichen Planus ; Melanoma ; Pityriasis ; Pityriasis Rosea ; Psoriasis ; Skin Diseases* ; Skin*