Aplasia cutis congenita is a rare disease presenting in the newborn infant as localized areas of skin defect. Its association with a number of congenital malformations is well documented. I observed a newborn infant with typical aplasia cutis congenita. The skin defect of my case was found on the posterior fontanelle without any congenital malformation. Diagnosis was made by history, clinical and histopathological findings.
Cranial Fontanelles ; Diagnosis ; Ectodermal Dysplasia ; Humans ; Infant, Newborn ; Leprosy* ; Lymph Nodes* ; Lymphocytes* ; Rare Diseases ; Skin

Leprosy has two polar types. The one tuberculoid leprosy (TL) is characterized by well preserved cellular immunity with a good prognosia and the other lepromatous leprosy(LL) shows no cellular immunity with a poor prognosis. The preaent study was designed to measure the activity of adenosine deaminase (ADA) in sera and erythrocytes of leprosy patients, as it's activities are known to be decreased in immune deficiency diseases. There were no significant differences in the erythrocyte ADA activities among normal subjects(9. 60+4. 43 units/1012 cells), TL patients (7. 12+2. 51 units/1012 cells) and LL patients(6. 96+0. 81 units/1012 cells), The ADA activities in sera of TL patients(20.15+2. 90 units/L) did not differ from those of normal subjects(20.44+ 2. 07 units/L), but the LL patients(17. 52+3. 30 units/L) showed a slightly lowered activity than those of normal subjects.
Adenosine Deaminase* ; Adenosine* ; Deficiency Diseases ; Erythrocytes* ; Humans ; Immunity, Cellular ; Leprosy* ; Leprosy, Tuberculoid ; Prognosis

Since Epsteins report in 1963, which identified topical corticosteroid therapy as a possible cause of striae formation, many adverse effects resulting from topical steroids therapy has heen observed in dermatological practice. In this study, 365 cases of side-effects with topical steroids in Department of Dermatology, Chonnarn University Hospital from 1972 to 1981 were analyzed, and the results obtained can be summerized as follows; 1. During this pericd, themean average percent of side effects with topical steroids was 0.85 of the yearly total patients and 12 different kinds of side-effects were found to be present in this study. 2. The annual rate of increase of side-effects with topical steroids (2.4%) was higher than that of yearly total patients (0.5%) and the annual frequency of the 12 side-effects was shown in Table 1. 3. The frequency of the 12 side-effects by age group was shown in Table 2. 4. The frequency of the 12 side-effects by season, sex and region was shown in Table 8. 5. The frequency of the 12 side-effects by lesion site was shown in Table 5. 6. T.he mean period of application with topical steroids was 4. 18 months and applied topical steroids were betamethasone-17-valerate(30.4%), fluocortolone (22.7%), hetamethasone dipropionate(12.9%), fluocmolone acetonide (9.0%) and prednisolone(8.8%) respectively. 7. The most frequent topical steroids and period of application causing each side-effects were as follows: betamethaaone-17-valerate for 1 month resulting in Steroid acne, betamethasone-17-valerate for 2 months resulting in Telangiectasia rubeosis et steroidica, triamcinolone acetonide for 1 month resulting in Perioral dermatitis and betarnethasone-17-valerate for 11 months resulting in Atropic striae.
Acne Vulgaris ; Dermatitis, Perioral ; Dermatology ; Fluocortolone ; Humans ; Seasons ; Steroids* ; Telangiectasis ; Triamcinolone Acetonide

A psoriatic patient may have rheumatoid arthritis, psoriatic arthritis(or both), osteoarthritis or gout. In so far as possible, each of these must be distinguished on clinical grounds with some help from laboratory tests. Psoriatic arthritis is very similar to rheumatoid arthritis but clinically, it is regarded as a unique disease entity, which is found in 1% to 32% of psoriatic individuals. We herein report two cases of psoriatic arthritis that are thought to be distal type and arthritis mutilans on the basis of clinical, serological and radiological features.
Arthritis ; Arthritis, Psoriatic* ; Arthritis, Rheumatoid ; Gout ; Humans ; Osteoarthritis ; Psoriasis

Leiomyosarcoma arising in the skin is rare tumor, and diagnosis usually is made microscopically. After local excision, these lesions recur in large proportion of pat ients. The authors herein report a 53-year-old male with leiomyosarcoma appeared in the skin of the right forearm and presenting as a dark reddish colored, 5*6cm in diameter, superficial ulcerated single firm nodule with intermittent pain. Histopathological examination showed poorly circumscribed tumor consisting of interlacing bundles of spindle shaped smooth muscle cells in the middle and lower parts of the dermis. The nuclei were hyperchromatic, large, vacuolated, and irregular in shape. Electron microscopic findings revealed cytoplasmic organelles such as rough endoplasmic reticulum and mitochondria of malignant smooth muscle cells in the paranuclear area, Characteristic subsarcoelmmal caveolae and dense plaque were noted and myofilaments were distributed in the peripheral cytoplasm. The tumor did not recur in 10 months' follow-up.
Caveolae ; Cytoplasm ; Dermis ; Diagnosis ; Endoplasmic Reticulum, Rough ; Follow-Up Studies ; Forearm ; Humans ; Leiomyosarcoma* ; Male ; Middle Aged ; Mitochondria ; Myocytes, Smooth Muscle ; Myofibrils ; Organelles ; Skin* ; Ulcer

BACKGROUND: It has been well known that patients with diabetes mellitus are prone to cutaneous infections, but whether superficial mycoses are truly more prevalent in diabetic patients is still controversial. OBJECTIVE: The purpose of this study was to determine the true prevalence of superficial mycoses in diabetic patients as comparing with non-diabetic, non-immunocompromised individuals. METHODS: We examined for evidence of superficial fungal disease in 137 diabetic patients and compared it with 146 non-diabetic non-immunocompromised ones. Potassium hydroxide preparations and fungus cultures were performed from all suspected lesions. RESULTS: Superficial mycoses were more common in diabetic patients(50%) than controls(31%) (p=0.0004), especially in the age group of 4th and 5th decades9p=0.011). The duration of diabetes mallitus or blood sugar levels were not significantly correlated with the prevalence of superficial mycoses.The organism most commonly isolated was Trichophyton rubrum in both groups(53% vs 79%) and Candida albicans was more prevalent in diabetic patients(34% vs 7%). More than 2 kinds of superficial mycoses were mose common in diabetic patients than controls(38% vs 16%). CONCLUSION: This study shows that the prevalence rate of superficial mycoses in diabetic patients is higher than that in controls and the in controls and the duration of diabetes mellitus or blood sugar levels were not significantly correlated with it.
Blood Glucose ; Candida albicans ; Diabetes Mellitus ; Fungi ; Humans ; Mycoses* ; Potassium ; Prevalence ; Trichophyton

No abstract available.
Dermatomyositis* ; Stomach Neoplasms*

BACKGROUND: Sunscreens have been used widely to prevent the photosensitive skin diseases, skin cancer, and skin aging. However, no sunscreen blocks all kinds of effects caused by ultraviolet light(UVL), and the effect of sunscreens on the impairment of immune function by UVL irradiation is controversial. OBJECTIVE: We try to evaluate the efficiency of sunscreens for blocking the depletion of LC induced by UVB irradiation. METHOD: The ATPase positive LCs were observed in the skin of hairless mice(Hr+/Kud) irradiated by UVB with or without topical application of sunscreens. Two commercially available sunscreens with respective SPF 8 and SPF 30 were applied to the dorsal trunk skin. The mice were irradiated with different increasing doses of UVB at a single time. RESULTS: The ATPase positive LCs in the irradiated dorsal and ear skin were significantly de-creased in densities according to the dosage, and apparently revealed a loss of their dendrites, granulation, and clumping from a UVB dose of more than 60mJ/Cm2. With both sun-screen treatment on the dorsal trunk before irradiation, the densities of LCs on the dorsal skin were significantly higher compared to the un-treated groups at all ranges of UVB doses in spite of a dose dependent decrease in their density. However there was no significant difference on their preventive effect between both sunscreens(SPF 8 and SPF 30) except at high UVB dos-es of more than 240mJ/Cm². CONCLUSION: The LC depletion induced by UVB can be partially protected through the topical application of a sunscreen at a UVB dose dependent fashion. However SPF(sun protective factor) dose not appear to be a good indicator for evaluating sunscreens immunologically.
Adenosine Triphosphatases ; Animals ; Dendrites ; Ear ; Langerhans Cells* ; Methods ; Mice ; Mice, Hairless* ; Skin ; Skin Aging ; Skin Diseases ; Skin Neoplasms ; Sunscreening Agents*

We herein report a 4-year-old boy with infantile digital fibrornatosis developing on the distal & middle phalanx portion of the right index & ring fingers, and the middle phalanx portion of the right middle finger, which started at the age of 5 months after birth. Biopsy specimen taken from the right middle finger showed the proliferating collagen bundles and fibroblasts in the dermis and eosinophilic cytoplasmic inclusions within the fibroblasts. Although we attempted to treat tbe patient with intralesional injection of triamcinolone acetonide suspension and cryotherapy 5 times biweekly, there was no signifit effect.
Biopsy ; Child, Preschool ; Collagen ; Cryotherapy ; Dermis ; Eosinophils ; Fibroblasts ; Fibroma* ; Fingers ; Humans ; Inclusion Bodies ; Injections, Intralesional ; Male ; Parturition ; Triamcinolone Acetonide

BACKGROUND: Immune tolerance induction (ITI) can reduce inhibitors against factor VIII concentrates by 70-80%. In this study, we elucidated the characteristics of inhibitors and attempted to determine the proper indications and timing for ITI. METHODS: Subjects included hemophilia A patients registered at the Korea Hemophilia Foundation from 1991 through 2014. Inhibitors were classified as persistent and transient. Patients were classified into groups according to peak inhibitor titer: low (<2 BU/mL), moderate (2 to <5 BU/mL), high (5 to <10 BU/mL), and very high titer (≥10 BU/mL). RESULTS: Overall, 350 (21.4%) of 1,634 hemophilia A patients developed inhibitors at least once. Of these, 100 (6.1%) and 250 (15.3%) patients developed persistent and transient inhibitors, respectively. For transient inhibitors, the median peak titer was 1.0 BU/mL, persistent for median of 11.0 months (10.0, 8.0, 13.0, and 19.0 months in the low, moderate, high, and very high titer transient inhibitor groups, respectively). Overall, 95.8% (215), 72.2% (17), 52.4% (21), and 21.7% (97) of patients in the low, moderate, high, and very high titer groups became inhibitor-negative spontaneously, without ITI. CONCLUSION: Given the spontaneous disappearance of inhibitors and high cost of ITI, it is worthwhile to postpone ITI for 11 months unless the peak inhibitor titer is greater than 10 BU/mL.
Factor VIII ; Hemophilia A* ; Humans ; Immune Tolerance ; Korea ; Longitudinal Studies