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

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*

Sarcomatous Intrahepatic cholangiocarcinoma is a rare but an aggressive variant of cholangiocarcinoma with a very poor prognosis. A 79-year-old man was admitted to our hospital because of incidentally found liver mass. Magnetic resonance imaging (MRI) revealed well-defined hypointense mass on T1WI and heterogeneous hyperintense mass on T2WI. Gd-EOB-DTPA enhanced study shows peripheral rim-like enhancement in arterial phase and progressive concentric filling of contrast in delayed phase. And mass shows significant enhancement in hepatobiliary phase. The pathologic diagnosis was intrahepatic cholangiocarcinoma with sarcomatous change.
Aged ; Cholangiocarcinoma* ; Diagnosis ; Humans ; Liver ; Magnetic Resonance Imaging* ; Prognosis

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

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

In Korea, Kerion celsi caused by T. verrucosum was first reported by Kim et al in the Honam province in 1986. Since then three mare cases have been reported in the Young Nam province. Herein present a case of kerion celsi caused by T. verrucosum from Chungcheong province. The patient, a 7-year old female, showed a 4x 4cm sized, oval, yellow crusted and boggy patch with alopecia on the scalp. She was living on a farm in a rural area of Chungcheong province. Cows on the farm also exhibited similar skin lesions on the buttock. The same T. verrucosum was identified by a characteristic colony morphology on Sabourauds dextrose agar and chains of chlamydoconidia with mycologic stain from scales of lesions of patient and cows. This skin lesion was successfully treated with griseofulvin.
Agar ; Alopecia ; Buttocks ; Child ; Female ; Glucose ; Griseofulvin ; Humans ; Korea ; Scalp ; Skin ; Tinea Capitis* ; Weights and Measures

OBJECTIVE: The study was made in order to inverstigate the clanical and mycological status of dermatophytosis in Chungchong province, incidence and friquency of disease, sex and month distribution of the disease, varieties of the causative organism. METHODS: We performed clinical and mycological studies of 86 cases of dermatophytosis which have showed positive on KOH examination and/or positved result in culture among out- patients of Dermatologic Clinics of Chungnam National Univesy during 7 years, from 1986 to 1993. RESULTS & CONCLUSION: 1. The ratio of male to female patients 330: 256 and make little ifference. 2. The incidence rate was bighest in the third decade(25.4%) 3. The seasonal prevalence of dermatophytosis was highest in the summer 4. The incidence of dermaiophytosis was T. pedis(30.2%), T. (22.9%), T. ungium(13.1%), T. corPoris(10.1%), T. capitis(9.0%) in decreasing order of freqercy 5. A mean positive rate for the KOH mount examination A. 5. 83.8%, T. 90.2%, T. corporis 89.8%, T. ungium 86.5% in decreasing order of frequency 6. A mean positive rate of culture was 77.1%; T. capities 94.3, . faciale 87.0%, T. cruris 84.3 %, T. cpor6 78.0% in decreasing order of frequency 7. T. rubrum was the mot common causative organism of cernatophytosis(69.5%), with T. mentagroPhyte(16.1%), M. canis(13.5%), M floccosum(0.7%), 4 gypseam(0.2%) presenting in decreasing order of frequency
Chungcheongnam-do ; Female ; Humans ; Incidence ; Male ; Prevalence ; Seasons ; Tinea*

BACKGROUND: Wound fluid is believed to contain growth factors produced by cells involved in the healing process. One of the beneficial effect of occlusive dreesing is the retention of released fluid from the wound, which is thought to accelerate wound healing. However, there was no report how suction-induced or cryo-induced bulla flicmight affect the healing of the wound itself. OBJECTIVE: Therefore, we have examined the effect of sucior-or cryo-induced bulla fluids on the in vitro growth of human dermal fibroblasts. METHODS: Suction-or cryo-induced bulla fluid were sterilely collected from the suction blisters and cryotherapy induced bulla, respectively. After seeding in optimal growth media on day 0, cultures of normal fibroblasts were supplemented with various concentrations of human serum, suction-induced bulla fluid, and cryo-induced bulla fiuid on day 2, 4, 6, 7, or 8. RESULTS: As determined by cell counts or 3H-thymidine incorporation, the effect of human serum were much more than those of suction-induced. Bulla fluid on the growht of fibroblasts. Although slight mitogenic effects were observed in the case of 5% cuyo-induced bulla fluid, 10% cryo-induced bulla fluid inhibited the growth of the fibroblasts as wello CONCLUSION: These results suggested that suction-induced bulla fluid was not a whole human serum but a diluted serous material having limited stimited stimulated effect to the growth of the fibroblasts, and cryo-induced bulla held the unknown inhibitory or toxic factors as well.
Blister ; Cell Count ; Cryotherapy ; Fibroblasts* ; Humans ; Intercellular Signaling Peptides and Proteins ; Suction* ; Wound Healing ; Wounds and Injuries

BACKGROUND: Acitretin, a synthetic retinoid, has been used to treat patients with psoriasis, Darier's disease, ichthyosis, keratosis pilaris etc. Some trials have suggested that oral acitretin may be useful for the treatment of warts. OBJECTIVE: The purpose was to determine whether oral acitretin is effective in the treatment of multiple and recalcitrant warts. METHODS: 25 patients with multiple and recalcitrant warts were enrolled. We administered acitretin, 30 mg daily in adults, and 0.5 mg/kg daily in children. At the end of the treatment, 21 patients were examined to determine the efficacy of the treatment. RESULTS: Of the 21 patients, 10 patients (48%) showed a complete response, 3 (14%) showed a moderate response, 4 (19%) showed a partial response, and 4 (19%) showed no response. Therefore, the patients showing more than a moderate response were 62% (13 of 21). An abnormal laboratory finding was not detected and adverse effects were common but tolerable in most patients. CONCLUSION: Oral acitretin may be useful for the treatment of extensive and reclacitrant warts, especially verruca plantaris.
Acitretin* ; Adult ; Child ; Darier Disease ; Humans ; Ichthyosis ; Keratosis ; Psoriasis ; Warts*

We present a case of generalized grannuloma annulare with perforating and subcutaneous forms which occurred in a 15-year-old girl. Two years ago, multiple umbilicated, match head to pea sized, erythematous papules were noted on the upper extremities. Then the patient also experienced crusted or ulcerated erythematous papular lesions on the lower legs. Recently, tender, subcutaneous nodules appeared on both soles. The biopsy specimens from the skin lesions of the hand and the sole showed typical findings of granuloma annulare in the dermis and the subcutis, respectively. On the histopathological examination of the calf lesion, palisading granuloma as well as transepidermally eliminating dermal collagenous and necrotic material were seen. The skin lesions were almost completely improved by treatment with dapsone, 100mg a day, for 8 weeks orally.
Adolescent ; Biopsy ; Collagen ; Dapsone ; Dermis ; Female ; Granuloma Annulare* ; Granuloma* ; Hand ; Head ; Humans ; Leg ; Peas ; Skin ; Ulcer ; Upper Extremity