Chronic mucocutaneous candidiasis (CMCC) is a general term used to denote a complex group of disorders characterized by a recurrent and persistent infection of the skin, mucous membranes and nails with organism of the genus candida, most frequently Candida albicans. It is often associated with an endocrinopathy and cell mediated immunopathy. CMCC is not a single disease entity, but rather a final common pathway for multiple predisposing abnormalities of the immune system that ranges from severe, life-threatening immunodeficiency syndromes to subtle deficiencies, especially of cell mediated immunity. Conditions that have been associated with CMCC include; candida esophagitis or laryngitis, endocrinopathies (usually hypoparathyroidism, hypadrenalism, hypothyroidism), circulating autoimmune antibodies, diabetes mellitus, vitiligo with antibodies to melanocytes, iron deficiency, chronic active hepatitis, pernicious anemia, malabsorption, alopecia totalis, dental enamel dysplasia, keratoconjunctivitis, pulmonary fibrosis, KED syndrome (keratitis, ichthyosis, and deafness), and recurrent pyogenic, viral or other fungal infections. When CMCC first appaears in adulthood, it is often associated with a thymoma. There is virtually no propensity for disseminated, visceral candidiasis. A suitable clinical classification of the major subtypes of CMCC was described by Lehner and Wells et al into six groups. Kirkpatrick et al found the mean age of onset of CMCC to be 3 years and both boys and girls are affected equally. We review CMCC and 5 reported CMCC cases in the dermatologic literatures in Korea.
Age of Onset ; Alopecia ; Anemia, Pernicious ; Antibodies ; Candida ; Candida albicans ; Candidiasis ; Candidiasis, Chronic Mucocutaneous* ; Classification ; Dental Enamel ; Diabetes Mellitus ; Esophagitis ; Female ; Hepatitis, Chronic ; Humans ; Hypoparathyroidism ; Ichthyosis ; Immune System ; Immunity, Cellular ; Iron ; Keratoconjunctivitis ; Korea* ; Laryngitis ; Melanocytes ; Mucous Membrane ; Pulmonary Fibrosis ; Skin ; Thymoma ; Vitiligo

Nevus sebaceus of Jadassohn has been referred to as a organoid nevus that classically evolves through three stages and may be associated with a range of skin tumors, and of these, syringocystadenoma papilliferum and basal cell carcinoma have been commonly reported to occur. The occurrence of keratoacanthoma is a very rare event in nevus sebaceus. Herein we report on a 23-year-old man with nevus sebaceus of the cheek. He represents a keratoacanthoma arising within nevus sebaceus.
Carcinoma, Basal Cell ; Cheek ; Humans ; Keratoacanthoma* ; Nevus* ; Nevus, Sebaceous of Jadassohn ; Organoids ; Skin ; Young Adult

Plexiform schwannoma is a relatively rare, benign peripheral nerve sheath tumor that can be located either in the deep soft tissues or in the dermis or subcutaneous tissue. This tumor may occur singly or as multiple lesions and may be localized to one anatomic site or diffusely distributed. Plexiform schwannoma should be differentiated with plexiform neurofibroma or other plexiform malignant tumors. We describe a case of a 6-year-old patient with multiple cutaneous plexiform schwannomas who had no other stigmata of neurofibromatosis 1 or family history suggesting a genetic disorder. The histopathological study revealed a tumor composed of multiple intradermal or subcutaneous interlacing and interconnecting fascicles and nodules that vary in size and shape. Characteristic Antoni A type cellular tissue showing frequent nuclear palisading and Verocay bodies were observed within well circumscribed elongated nodules.
Child ; Christianity ; Dermis ; Humans ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatosis 1 ; Peripheral Nerves ; Subcutaneous Tissue

To evaluate whether mast cells are involved in developing pathologic feature of psoriasis, 60 biopsy specimens of patients with psoriasis were analyzed. They had not been treated for at least 1 week before skin biopsy. Histological findings in early and fully developed lesions and numbers of mast cells in their dermal papillae were investigated. The results were as follows: 1. In epidermal changes of psoriatic lesions, parakeratosis and acanthosis revealed different findings between early lesions and fully developed lesions. While early lesions revealed mounds of parakeratosis and mild to moderate acanthosis, fully developed lesions revealed confluent parakeratosis and moderate to severe acanthosis. In dermal changes of psoriatic lesions, papillomatosis revealed different findings between early developed lesions and fully developed lesions. While early lesions revealed normal to moderate papillomatosis, fully developed lesions revealed moderate to severe papillomatosis. 2. Degree of acanthosis is related to the degree of papillomatosis. The more increase in the degree of acanthosis, papillomatosis, and parakeratosis, mast cell numbers in dermal papillae were more increased. 3. Mast cell numbers in dermal papillae were more increased in fully developed lesions than early lesions. 4. These findings suggest that mast cell may play an active role in developing pathologic finding of psoriasis.
Biopsy ; Humans ; Mast Cells* ; Papilloma ; Parakeratosis ; Psoriasis* ; Skin

No abstract available.
Acute Kidney Injury* ; Humans ; Pancreatitis*

We report a case of metastatic carcinoma of the skin in 62-year-old male who showed multiple nodules on the anterior chest, scalp, face and left axilla for 2 months duration. He suffered from lower abdominal discomfortness for 6 months. Histopathologic findings revealed atypical cell nests and some tubular or glandular formation in the dermis and subcutaneous fat tissue. Material in the lumen showed diastase resistant and PAS positive, and alcian blue positive at pH 2.4, but negative pH 0.4. So it represented sialomucin. We did not find primary site of malignant tumor. In view of clinical and histopathological findings, it was considered to be originated from the large intestine.
Alcian Blue ; Amylases ; Axilla ; Dermis ; Humans ; Hydrogen-Ion Concentration ; Intestine, Large ; Male ; Middle Aged ; Scalp ; Sialomucins ; Skin* ; Subcutaneous Fat ; Thorax

No abstract available.
Cyclotrons*

Sustained abnormal liver function is closely associated with chronic hepatitis in Korea with high prevalence of hepatitis B infection. Follow up about AST and ALT on 839 adults(male 727, female 112) was executed with interval of at least 6 months for 2 years to evaluate the factors contribute to the progression of chronic hepatitis. HBsAg, HBsAb, anti-HCV, body mass index(BMI), total cholesterol, fasting blood sugar(FBS), Clonorchis sinensis in stool(Cs), fatty liver on abdominal sonogram and alcohol habit were also checked. We defined abnormal liver function as both of AST 35 U/L and ALT 50 U/L. As the result of first time liver function test(LFT), the frequency of abnormal liver function was 13.6%(114/839). The positive rate of HBsAg and HBsAb was 7.0% and 73.9%, respectively, and the positive rate of anti-HCV and Cs was 0.7% and 1.3%. As the result of the follow up, 6.2%(52/839) in total has a tendency of chronic hepatitis with sustained abnormal LFT. Among abnormal LFT, 45.6%(52/114) was sustained LFT abnormality, and 12.8%(93/725) was aggravated LFT abnormality from normal LFT at the first check. From these results, male and hepatitis B infection were evaluated as important factors for the progression of chronic hepatitis. HBsAb considered that has a protective effect to the progression of chronic hepatitis. Therefore, even though no symptoms or sign, periodic examination for hepatitis B marker and LFT is important to control the chronic hepatitis, especially for male. Furthermore, because only 25%(13/52) of sustained abnormal LFT was HBsAg positive, it suggests that further study for evaluating the factors except hepatitis B infection would be needed for the control of chronic hepatitis.
Cholesterol ; Clonorchis sinensis ; Fasting ; Fatty Liver ; Female ; Follow-Up Studies ; Hepatitis B ; Hepatitis B Surface Antigens ; Hepatitis, Chronic* ; Humans ; Korea ; Liver ; Male ; Prevalence ; Transaminases*

Xanthoma tendinosum is a hereditary lipid storage disease marked by formation of papules or nodules in the tendon, especially the extensor tendon of the hand and feet and the Achilles tendon. Xanthoma of the Achilles tendon is a rather rare, interesting orthopaedic condition that has important ramification in internal medicine and dermatology because the lesion is associated with a specific disturbance of lipid metabolism with elevated blood cholesterol level. Author present a case of bilaterally involved Achilles tendon Xanthoma. We performed subtotal resection with gastrocnemius fascial transfer on one side and total resection with peroneus brevis tendon transfer on the other side.
Achilles Tendon ; Cholesterol ; Dermatology ; Foot ; Hand ; Internal Medicine ; Lipid Metabolism ; Tendon Transfer ; Tendons ; Xanthomatosis

Cardiopulmonary bypass (CPB) in children is associated with the accumulation of body water after cardiac operation, as a consequence of an inflammatory capillary leak. Following work by Elliott in 1991, modified ultrafiltration (MUF) was introduced after bypass as a means of hemoconcentrating patients and a potential way of removing water from the tissues. We have carried out a prospective randomized study of 20 children undergoing open heart surgery, comparing MUF with nonfiltered controls. MUF was carried out for a mean of 18.9 minutes after completion of CPB to a hematocrit of 37.1% (mean). The mean water volulme removed by the ultrafiltration was 38.4 ml/kg and the mean blood volume retransfused from the oxygenator during the ultrafiltration was 32.1 ml/kg. Fluid balance, hemodynamics, hematocrit, osmolarity and dosage of drug treatment were recorded for 4~12 hours postoperatively. The results were analyzed using Student t-test and ANOVA, comparing controls (n=10) to MUF (n=10). Blood loss (ml/kg/24hr) was 14.5 (mean) in MUF versus 13.7 in controls; blood transfused (ml/kg/24hr) 6.6 in MUF versus 15.2 in controls; plasma transfused (ml/kg/24hr) 65.7 in MUF versus 59.6 in controls. There was rise in arterial blood pressure and hematocrit during MUF. Percent rise of systolic blood pressure was 28.8% in MUF versus 18.7% in controls (p=0.366); percent rise of diastolic blood pressure was 28.8% in MUF versus 8.5% in controls (p=0.135); and percent rise of mean blood pressure was 36.2% in MUF versus 8.2% in controls (p=0.086). Percent rise of hematocrit was 40.0% in MUF versus 23.5% in controls (p=0.002). There was no significant difference in the inotropic requirement and the postoperative serum osmolarity between two groups. The number of days on the ventilator, the duration of stay in the intensive care unit, and the postoperative hospital stay were not significantly different between the two groups.
Arterial Pressure ; Blood Pressure ; Blood Volume ; Body Water ; Capillaries ; Cardiopulmonary Bypass ; Child ; Heart* ; Hematocrit ; Hemodynamics ; Humans ; Intensive Care Units ; Length of Stay ; Osmolar Concentration ; Oxygen ; Oxygenators ; Plasma ; Postoperative Period ; Prospective Studies ; Thoracic Surgery* ; Ultrafiltration* ; Ventilators, Mechanical ; Water-Electrolyte Balance