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

We report a case of eosinophilic pustular folliculitis in a 42-year-old woman who had atypical clinical findings with moderately pruritic erythematous follicular papules and pustules on the face and neck. Histopathologically miny eosinophils and neutrophils infiltrated around the follicles, within the follicles and around tbe vessels. The follicular walls showed spongiosis. The differential count of eosinophils in seram was 1.9%. The patient responded well to systemic steroid and dapsone.
Adult ; Dapsone ; Eosinophils* ; Female ; Folliculitis* ; Humans ; Neck ; Neutrophils

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

Hemophilia is the most common coagulation disorder. It has a long history. Hemophilia A is caused by FVIII gene mutation, and hemophilia B by FIX gene mutation. Those genes are located on X chromosome long arm. Bleedings in hemophiliacs predominantly occur in joints and muscles. Because those site are insufficient in tissue factor to induce hemostasis. Among joints knee, ankle and elbow are most frequently affected because their synovial structure is vulnerable to injury compared to other joints. Hemophilia is diagnosed with factor assay. Severe hemophilia is below 1% of FVIII : C, moderate between 1% and 5%, mild over 5%. Carrier detection and prenatal diagnosis have been conducted with RFLP-based linkage analysis and DNA sequencing. Mainstay of treatment is factor replacement therapy so far. Bleedings can be controlled by infusion of factor concentrates. Hemophilc arthropathy and muscle contracture are representative sequelae. Complications of facotor replacement therapy are inhibitor development and infections. Hemophiliacs with inhibitor should be managed with large dose factor concentrate, bypassing agent, ITI and immunosuppression. Ultimately, hemophilia could be cured by gene therapy.
Ankle ; Arm ; Contracture ; Diagnosis ; Elbow ; Genetic Therapy ; Hemophilia A* ; Hemophilia B ; Hemostasis ; Immunosuppression ; Joints ; Knee ; Muscles ; Prenatal Diagnosis ; Sequence Analysis, DNA ; Thromboplastin ; X Chromosome

No abstract available.
Knee* ; Ligaments*

No abstract available.
De Lange Syndrome*

We herein presented a case of persistent variant of Grovers disease in a 83-year old male. He had a rather extensive eruption of erythematous scaly papules, which had been profusely grouped but discretely distributed over 2 years or more. The histopathologic findings of two lesions in the same patient revealed mixture of various histologic patterns, patterns resembling Dariers disease, pemphigus vulgaris, Hailey-Hailey disease and spongiosis. The ultrastructural findings of Darier pattern should be essentially similar to those of Dariers disease and also be common to those observed in the early stages of other causes of focal acantholytic dyskeratosis. The patient had been treated with topical and intralesional steroid and oral 13-cis-isotretinoin, however, the lesions had been persisted only with partial improvement of clinical appearance and reduction of pruritus.
Aged, 80 and over ; Darier Disease ; Humans ; Male ; Pemphigus ; Pemphigus, Benign Familial ; Pruritus

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*

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