Using a paradigm similar to the one employed by Crowe et al., this study was undertaken to determine the genetic characteristics of multiple neurofibromatosis in Korean people. Thirty three subjects were used in this study. All subjects were identified through an exarnination of the records of all patients who had visited the Department of Dermatology of Chonnam Medical University Hospital between 1967 and 1976. Relatives of subjects were examined whenever possible, The results of the study projected that the total number of persons in Chonriarn Province afflicted with multiple neurofibromatosis lies sornewhere between 880 and 16,500. Crowe estimated that between 2500 and 3300 patients could be found on Michigans lower peninsula. Sixty percent of this studys subjects presented as sporadic patients, a condition thought to be the resu.lt of a genetic mutation. They had no blood relatives who demonstrated the disease. Fifty percent of Crowes subject were found to be sporadic patients, and a similar study by a Japanese investigator revealed a sporadic patient rate of sixty percent. In this study, the rernaininp 40% of the subject group conformed to the expected genetic frequency, thus pointing toward the fact that rnultiple neurofibrornatosis is a highly penetrant autosomal dorninant disease. This was demonstrted by the fact that, of the 79 offspring bad by this group, 82 persons demonstrated multiple neurofibromatosis. This study also suggested somatic mutation in 3 of the 20 sporadic patients. Because this nutation did not occur at the germinal level, the patient is much less likely to transmit this disease to his offspring, One patient showed the usual clinical and family traits but the chromosome analysis was negative. Genetic issues associated with multiple neurofibromatosis are also discussed in this sturly.
Asian Continental Ancestry Group ; Crows ; Dermatology ; Humans ; Jeollanam-do ; Michigan ; Neurofibromatoses* ; Research Personnel

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

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

A case of hypohidrotic ectodermal dysplasia was seen at the Dermatological Clinic of Chosun Univeraity Hospital, with a classical symptom triad consisting of hypohidrosis to anhidroais, hypotrichosis and hypodontia, and characteristic facial appearance. He was a 20 year-old male patient who presented, in addition, milium-like papules of sebaceous hyperplasia located on the nose and cheeks, supernumerary nipples of right breast, ceruminosis since childhood, and absence of apocrine glands on the axilla and pubic area, all of which are not frequently observed in hypohidrotic ectodermal dysplasia. Dermatoglyphics of this patient revealed that the axial triradius of the left palm was t, its atd angle was 42 degree, and the total ridge count of finger print was 5. This patient has no relatives showing the symptoms of hypohidrotic ectodermal dysplasia and the genetics was discussed in relation to this disease.
Anodontia ; Apocrine Glands ; Axilla ; Breast ; Cheek ; Dermatoglyphics ; Ectodermal Dysplasia 1, Anhidrotic* ; Fingers ; Genetics ; Humans ; Hyperplasia ; Hypohidrosis ; Hypotrichosis ; Male ; Nipples ; Nose ; Young Adult

Daily topical sequential triple therapy(tretinoin, betamethasone valerate and hydroquinone), as proposed by Kligman et al and Gano et al, has been performed on 29 Korean female patients with melasma. After 4 weeks treatment with 2% hydroquinone cream, only three of eight patients (37. 5%) showed good to excellent results. However, after 4 weeks treatment with 4% hydroquinone cream, twelve of twentyone patients (57. 1%) showed good to excellent results and its therapeutic effects appeared more rapidly than the former group. Overall, after 4 weeks treatment. 96.6% of the total group showed fair to excellent results of which 57. 7% had a good to excellent rating. The fair-com- plexioned Koreans had a better response than dark-complexioned.
Betamethasone Valerate* ; Betamethasone* ; Female ; Humans ; Melanosis* ; Tretinoin*

A case of Ramsay Hunt syndrome, though more often seen at the ENT clinic because of the patients' concern with symptoms in the ear, was seen and treated at the Dermatologic Clinic of Chosun University. A 29 year old male patient presented earache, pinhead to rice sized erythematous vesicular eruption on the external auditory canal, auricle and superior auricular area, and facial paralysis, all involving the right side of the face, duration of which were 7 days, 5 days and 1 day respectiveIy. High doses of prednisolone alone made it possible to remit the otalgia, control the vesicular eruption and resolute the facial paralysis almost completely within 18 days in the order of their appearance, indicating that this is one of the more easily treated diseases for dermatologists, The 22 cases of this syndrome reported in Korean literature were reviewed and the conceptual development of its pathogenesis and definition was described.
Adult ; Ear ; Ear Canal ; Earache ; Facial Paralysis ; Herpes Zoster Oticus* ; Humans ; Male ; Prednisolone

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

In a large proportion of patients with chronic urticaria, at present, the definite tiology can't be found. There have been occasional attempts to identify the aIIergic causes uaing allergic skin tests, only ta suggest the Candicla albicans and. some food yeasts ae probabIe causes. Thia atady was designed to investgate how often patients with chronic urticaria from Chonnam Province react positively to skin tests with extracts of 42 common. allergene in the standard group Al-13 produced by Bencard Ltd. in England. The authora alao surveyed the clinical characteristics of 63 patients with chronic urtimria of more than 3 months duration, who bad visited the skin clinic of Chosun Univeresity HcepitaI from May. 1977 throug 3. Apr. 1979. The results were summarized as follows; 1. Among 63 patients, the maIe to female ratio was 1: l.2, and the peak incidence was in the 20s and 30s. 2, In regard to the duration of chroic urticaria, the highst frequency waa below one year duration. 3. Among 60s of the pa,tients who experienced seasonal changes, 32.4% had suffered aggravation during the winter. 4. The most common diseases combined with chronic urticaria were allergic rhinitis and atopic dermatitis, occuring in 9.5% of all subjects. 5. Of thase examined, 69.8% showed positive reactions to the prick test with the peak incidence in the 20s and 30s. 6. Among 42 allergens used far the prick test, 28 allergens turned out to be possibly casal agents, the rnost frequent group of allergens involved were grain du sts (27.1%), animal hairs(21.8%) and moulds(16.5%) in that order of frequency and occupying over 65% of the total positive reactions. And the most frequently involved specific allergens were hay dust, mixed grain dust straw dust and tree in that order of frequency. Moreover, 90 of the involved allergens were inhalants. 7. In teen-agers, the reactions to grain dusts, animal ha.irs, rnoulds and pollens were the rnost frequent while reactions to food allergens were most frequent in the 20s, and cotton and house dust are most frequently involved in tbe 40s. 8. Multiple sensitivity was not:d in a majority of cases and the most frequent combination was 2 to 5 allergens. 9. The rnost common abnormal hematologic finding in chronic urticaria was eosinophilia.
Allergens ; Animals ; Edible Grain ; Dermatitis, Atopic ; Dust ; England ; Eosinophilia ; Female ; Humans ; Incidence ; Jeollanam-do ; Pollen ; Rhinitis ; Seasons ; Skin ; Skin Tests ; Urticaria* ; Yeasts