Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected. Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected. Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected.
Consanguinity ; Family Characteristics ; Fertility ; Genes, Dominant ; Humans ; Incidence ; Inheritance Patterns ; Neurofibromatoses* ; Neurofibromatosis 1 ; Wills

While for the last thirty years Dapsone (4,4, diaminodiphenyl sulfone; DDS) has been the chemotherapeutic treatment of choice in the management of leprosy, other non-sulfone compounds have been used when patients have shown either sulfone resistance or sulfone sensitivity. Unfortunately, however, there have gradually appeared a significant number of dapsone resistant and non-sulfone resistant patients (i. e., patients resistant to the conventional chemotherapeutic management of leprosy), thus necessitating the synthesis of additional antileprotic medication. At present, it appears that Lamprene (Clofazimine) is the most adequate preparation for the treatment of sulfone and/or other anti-leprotic drug resistant cases, as well as reactive states. The work of Browne and Hogerzeil in 1962, and subsequent studies by ether workers, have demonstrated lamprenes anti-leprotic and anti-inflamatory effects. The drug has also been need successfully in the management of the reactive patient. However, as its most untoward side effect, the drug causes an unsightly darkening of the skin in those areas where the concentration of M. leprae is greatest. Because the literature provides only sparse data on the effect of lamprene on the morphological (MI) and bacteriological (BI) indices of bacteriologically open patients, the authors undertook the following study: Eighteen dapsone resistant patients, two of whom were in lepra, reaction, received a daily dose of 100mg. of lamprene during a period. ranging from 4 to 22 months. Patients were kept under close clinical observation and bacteriological samples were taken at an average of three month intervals from eight different sites on the body, All subjects were in residence at the National Leprosy Hospital of Korea on Sorok island. The study yielded the following results: 1) Within 3 to 8 months after the administration of lamprene, the MI decreas d to the base line in all patients save one. 2) In the short term administered group (less than 10 months), 6 of 1R patients showed a BI increase in inverse proportion to an MI decrease during the initial stage of lamprene administration. However, the BI began to decrease between the 4th and 5th months of treatment. Of the remaining 7 patients, all showed a decrease in both BI and MI. 8) In the long term administered group (more than 10 months), the BI, an indicat- or in the evaluation of long term administration, gradually decreased in 4 of 5 patients. In the remaining patients the BI increased. The authors regard the inverse relationship between the BI and MI as the result of the increment of bacilli secondary to the destruction of M. leprae by lamprene. That groups showed a decrease in both BI and Ml is interpreted as lamprenes biochemical intervention so as to render M. leprae more susceptible to phagocytosis. While. no ready explanation can account for the single case in which the BI increased and the MI also increased, the pos. ibility that there might be a strain of M. leprae resistant to lamprene must be ruled out. thus, given the above results, the authors conclude that lamprene is a valuable antileprotic drug not only for DDF>resistant patients but also for patients in lepra reaction. Moreover, this drug seems to find its best setting in the leprosarium where the untoward side effect of darkened skin does not in any way diminish the patients social relationships.
Clofazimine* ; Dapsone* ; Ether ; Humans ; Korea ; Leprosy ; Leprosy, Lepromatous* ; Phagocytosis ; Skin

No abstract available.

Primary cutaneous monomorphous lymphoma is rare compared to the more usual involvement of skin secondary to internal monomorphous lymphoma. The histopathological diagnosis of the primary cutaneous monomorphous lymbhoma (PCML) requires differentiation from cutaneous lymphoid hyperplasia. The authors observed 3 cases of PCML. Case 1 was 21 year-old woman, who had an initial lesion on her left arm. Histopathologically it was diagnosed as poorly differentiated. lymphocytic lymphoma and was treated by surgical excision. Six months after onset she developed new lesions on her skin elsewhere, histopathologically diagnosed as well differentiated lymphocytic lymphoma. She died of infiltration of the bone marrow 19 months after the initial onset, even though combination of radiotherapy and chemotherapy resulted in clinical improvernent. Case 2 was a 70 year-old woman having an unusual cutaneous manifestation of an adult palm sized ulcerative, indurated tumor on her right forearm, histopathologically diagnosed as histiocytic lymphoma. There was no evidence of extracutaneous in volvement except right axillary lymphadenopathy. Case 3 was 72 year-old man, who had a clinical manifestation to that of case 2, histopathologically diagnosed as histiocytic lymphoma and received radiotherapy with good improvement. The patient did not show any evidence of extracutaneous involvement five months after the onset.
Adult ; Aged ; Arm ; Bone Marrow ; Diagnosis ; Drug Therapy ; Female ; Forearm ; Humans ; Hyperplasia ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Large B-Cell, Diffuse ; Radiotherapy ; Skin ; Ulcer ; Young Adult

Papulonecrotic tuberculid (PNT) and Pityriasis lichenoides et varioliformis acuta (PLEVA) have been in controversy in their pathogeneses and entity itselves. Authors reviewed litera,tures of the two dermatoses with observation of nine casea of PNT from 1979 to 1981 and evaluated whether PNT could be also classified as PLEVA. The results were a,s follows: 1. By the review of literatures PNT and PLEVA showed great similarities in their clinical aspects including shape of cutaneous lesions, course, favorite age and subjecive symptoms. Histopathological overlappings were also found. 2. Of the nine cases of PNT authors observed, seven cases were histopathologically PLEVA and four cases could be thought PLEVA also clinically. But way six cases were treated by anti-tuberculous drugs. 3. The incidence of other tuberculous signs and results of tuberculin skin tests were all significant in the nine cases above. With the above observations it seems that PNT is one of the causes of PLEVA and thus could be a kind of PLEVA.
Child* ; Dermatitis* ; Humans ; Incidence ; Pityriasis Lichenoides ; Skin Diseases ; Skin Tests ; Tuberculin ; Tuberculosis, Cutaneous

It has been known that osseous stability of the hip is dependent upon the inclination, anteversion and depth of acetabulum and also upon neck-shaft angle and anteversion of femur. Acetabulum enlarges from two different growth centers; one from the concave articular surface cartilage and the other from the triradiate cartilage. The former contributes primarily to the thickness of acetabular wall, where as, the latter to the central enlargement of the acetabular socket. Additionally the shape of acetabulum can be changed by deformity of the femoral head and neck, which can be explained by Wolff's and Hueter-Volkmann's laws. Yet there are still many factors to be clarified by clinical researchers in the future Authors in this experiment tried to clarify the role of triradiate cartilage and greater trochanteric growth plate upon the development of acetabulum. The material used for this study consiated of 30 rabbits of both sexes, aged 8 weeks. Animals were divided into 3 groups, and each group consisted of 10 rabbits In group I. hip was not injured and used as a Control: in group II, right greater trochanter with its growth plate was destructed completely; in group III, triradiate cartilage of right side was destructed. Subsequent to injury of Y cartilage ot greater trochanter with its growth plate, radiographs of the pelvis including hips were obtained weekly on each rabbit over 6 weeks period. and to evaluate the effect of injury upon the hip development acetatular angle, C-E angle, and acetabular depth and width were measured. The following results were obtained; 1. The shape of acetabulum of rabbits have showe remarable developmental changes until the 10 weeks of age after birth, but the changes became less remarkable by age of the 14 weeks. Thereafter it remained unchanged. 2. In group II, at the age of 14 weeks, compared with the values of the normal hip, the acetabular angle showed an increase of 1.9 degrees, and the center dege angle and acetabular depth showed a decrease of 3.21 degrees and 0.43mm respectirely. 3. In group III, at the age of 14 weeks, compared with the values of the normal hip, the acetabular angle increased by 18.9 degress while the center-edge angle decreased by 22.8 degrees. The acetabular depth showed a decrease of 1.81mm and the acetabular width showed an increase of 0.73mm. These observations suggest that the development of acetabulum depends largely upon the growth of triradiate cartilage, and also is affected secondarily by valgalization of femoral neck which is resulted by destruction of greater trochanteric growth cartilage.
Acetabulum ; Animals ; Cartilage ; Congenital Abnormalities ; Femur Neck ; Femur ; Growth Plate ; Head ; Hip ; Jurisprudence ; Neck ; Parturition ; Pelvis ; Rabbits

Recently the frequency of Microsporum canis, as a causative dermatophyte of tinea capitis, is increasing in our country. We herein report a case of tinea capitis caused by Microsporum canis in an eight-day-old infant. Infection was contracted, presumably, from a kitten who had tinea corporis caused by the same species of Microsporum and who lived in the patients house. It is extremely rare for tinea capitis to occur in the neonatal period. Although only one case had been reported in the foreign literature, to our knonwledge, a case has never been reported in Korea. We believe that this patient is the youngest patient with Miicrosporum canis infection of the scalp yet reported, and it is also of interest that he has atypical lesions with black-dot-like non-inflammatory alopecic patches on the scalp.
Arthrodermataceae ; Humans ; Infant ; Korea ; Microsporum ; Scalp ; Tinea Capitis* ; Tinea*

No abstract available.
Thanatophoric Dysplasia*

We observed allergic cutaneous reaction to vitsmin K, injection in 3 patients who developed pruritic erythematous indurated plaques at the sites of intramuscular injection and leakage sites of vitamin K1 preparation into dermis during IV injection 12 to 23 days after injection. Intradermal tests with vitamin K1,K3 and their placebos performed on 145 healthy medical students revealed positive skin reaction in 13 students 7 to 22 days after injection similar to the original 3 cases. Five students also showed the same reaction to vitarnin K1 placebo, however, a more severe reaction to vitamin K1 preparation than its placebo in 3 of them, indicating that the index of cutaneous sensitivity of vitamin K1 lies somewhere between 5. 5,% and 8. 9%. There was no cross sensitivity between vitamin K1 and K3 We consider these reactions to be of allergic nature.
Dermatitis* ; Dermis ; Humans ; Injections, Intramuscular ; Intradermal Tests ; Placebos ; Skin ; Students, Medical ; Vitamin K 1* ; Vitamins*

No abstract available.
Fibrosis*