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

Dermoid cyst is rare developmental anomaly with sequestration of embryonic closure line, but there is no suggestian that such an anomaly is inherited. The patients were 41 year old mother and 10 year old daugliter whose skin lesions have been noticed since puberta.lstae in the mother and since birth in the daugther It was interesting for those lesions to be appeared typically on the superior orbital area.
Adult ; Child ; Dermoid Cyst* ; Humans ; Mothers* ; Nuclear Family* ; Orbit ; Parturition ; Skin

Sole prints were analyzed from one thousand normal volunteers (500 males and 500 females) to establish dermatoglyphics of Korean people, and the following resuIts were obtained: The triradius P was present in 49.1% of the right soles and 40.6% of the lefts, or 44.9% of the entire series. The fibular radiant of triradius P was directed into the fourth interdigital intervals in 54.9% of the soles, and the next common terrninations were in the digital triradius c 15.3%, the third interdigital interval, 12. 5% and distal portion of the fibular border, 9.4%. In right soles the tendeney was for the radiant to extent farther tibialward than in lefts, where the inverse tendency was reflected in sharp rise in the number of endings on the fibular borders and decrease of endings tibialward. The distal radiant of triradius P terminated in the first interdigital interval, 46.5%, the interdigital triradius a, 32. 5%, and the second interdigital interval,. 20.5%, and rarely the distal radiant fused with digital triradius, 0.6% Of the alignments of ridges of the soles, 72.7;. of the soles exhibited transvcrsely coursing ridges in the mid-region, and the remainder were slight fibuloproximal slants,Grade 2) 18.2%, and slight fibulodistal slants (Grade 4) 8, 7%. There was a tendency of right soles to favor the fibulo-proximal slant, and of lefts, the fibulo-distal sIant. In the calcar region 44.1% of the soles presented a slight fibulo-distal slant, and the next cmmon direction was transverse, 37.4% Right soles tended more to the transverse alignment, while lefts displayed a ... (continue)
Dermatoglyphics ; Healthy Volunteers ; Humans ; Male

The accuracy and sensitivity of both direct and indirect immunofluorescence microseopy in diagnosing chronic bullous dermatoses were evaluated and compared in 11 cases of six different disease entities(pemphigus vulgaris, pemphigus vegetans, bullous pemphigoid, linear IgA bullous dermatosis, familial benign chronic pemphigus), which had been diagnosed clinically and by routine histopa thological studies. And the results obtained were as follows: 1) In 4 cases of bullous pemphigod, the direct IF of perilesional skin showed linear deposition of IgG and C2 along basement membrane zone(BMZ), whereas the indirect IF revealed negetive findings. 2) In 2 cases of pemphigus vulgaris, the direct IF showed deposition of IgG and C2 in intercellular substance(ICS) of perilesional epidermal tissue, and the indirect IF revealed auto-antibody to ICS(1:320) in one case. 3) In 2 cases of linear IgA bullous dermatosis, which were diagnosed as dermatitis herpetiformis by clinical and routine hisopathological findings, the direct IF of perilesional and uninvolved skin manifested linear deposition of IgA, IgM, C3 and F along BMZ, However, no immunofluorescence was detected by the indirect IF. 4) In a case of chronic bullous dermatosis of childhood, the direct IF of perilesional skin showed linear deplosition of IgA and IgM along BMZ. 5) In a case of pemphigus vegetans, the direct IF revealed no specific findings, while the inderect IF disclosed auto antibody positive to ICS(1:40). 6) In a case of familial benign chronic permphigus, no immunofluorescence was found by direct IF. These results indicate that both direct and indirect immunofluorescence micros-copy are valuable in diagnosing chronic bullous dermatoses and in understanding their immune pathogenesis.
Basement Membrane ; Dermatitis Herpetiformis ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Linear IgA Bullous Dermatosis ; Pemphigoid, Bullous ; Pemphigus ; Skin ; Skin Diseases ; Skin Diseases, Vesiculobullous*

We herein described a female newborn baby who, on the day of birth, exhibited widespread discrete reddish purple colored, round and slightly hyperkeratotic papules measuring about 4 mm in size, the biopsy specimen of which showed the histologic findings of histiocytosis X. The skin lesions resolved spontaneously within a week. The following diseases were discussed as possible diagnoses; histiocytosis X, generalized eruptive histiocytoma, juvenile xanthogranuloma, congenital self-healing reticulohistiocytosis and xanthoma disseminaturn. Since the current case didn't fit well any of them, we proposed a new descriptive term for this : Histiocytoma Papulosa Benigna Congenita.
Biopsy ; Diagnosis ; Female ; Histiocytoma* ; Histiocytosis, Langerhans-Cell ; Humans ; Infant, Newborn ; Parturition ; Skin ; Xanthogranuloma, Juvenile ; Xanthomatosis

Gamma-glutamyl transpeptidase (GGTP) activity was measured in the homogenate of penile foreskin, using y-glutamyl-p-nitroanilide, as a substrate, and it was found that (GGTP) activity was present in the epidermis and dermis, being more active in the former. The optimum pH for the enzyme was 8.5 - 9.0 in Tris buffer, which was similar to those of the rat kidney and human serum enzymes. It was also revealed that glycylglycine was the most effective activator of the enzyme and some activation was also observed in the presence of L-glutamine. But L-rnethionine, L-homoserine, L-glutamic acid, L-arginine, L-aspartic acid, glycine and L-valine inhibited the activity, suggesting that these amino acids do not act as acceptors of p-glutamyl moiety. The enzyme was remarkably inhibited by bromosulphalein, oxidised gluta,thione, and by L-serine in the presence of borate, and the inhibitions were more severe than is the case with the rat kidney and human serum enzymes.
Amino Acids ; Animals ; Arginine ; Aspartic Acid ; Dermis ; Epidermis ; Foreskin ; gamma-Glutamyltransferase ; Glutamic Acid ; Glutamine ; Glycine ; Glycylglycine ; Humans ; Hydrogen-Ion Concentration ; Kidney ; Patient Acceptance of Health Care ; Rats ; Serine ; Skin* ; Tromethamine ; Valine

Leprosy is an infectious diseases caused by Mycobacterium leprae. It is considered to be manifested in person with an impaired. immune system and is divided into two polar forms; the first being tuberculoid leprosy(TL) with nearly norrnal cell-mediated immunity(CMI) and the second heing lepromatous leprosy(LL) with deficient CMI. Adenosine deaminase(ADA) is an enzyme concerned with intermediary purine metabolism, which is known to be deficient in case of immunological dysfunction. To find out if there is any ADA deficiency in leprosy, the ADA activities in the lymphocytes of leprosy patients were compared with those of normal ones. The ADA activities in lymphocytes of normal subjects, TL patients and. LL patients were as follows; ID. 36-I-l. 90 units/10cells, 6. 35+0. 86units/10'cells and 4. 58+0. 52units/IO'cells respectively. The ADA activities in lymphocytes were revealed to be significantly different between normal subjects and LL patients(p<0. 01) and also between normal subjects and TL patients(p<0. 05). The lowered ADA activities in lymphocytes of leprosy patients, particularly in lepromatous leprosy, suggests a similar role in ADA for immunological response as demonstrated in severe combined immunodeficiency diseases.
Adenosine Deaminase* ; Adenosine* ; Communicable Diseases ; Humans ; Immune System ; Leprosy* ; Leprosy, Lepromatous ; Lymphocytes* ; Metabolism ; Mycobacterium leprae ; Severe Combined Immunodeficiency

Twenty six cases of Exfoliative dermatitis were found during the years from July of 1967 to June of 1972 at the department of dermatology of Chonnarn University Hospital, and the cases of the disease were studied for ciinical signs and symptoms, etiological factors, laboratory findings and responses to the treatment. The incidence of the disease to total number of skin patients in the years: 0. 2%, mean age distribution: 41. 9 years old, and the most cornmon etiological factors: drugs such as 4. 4-diaminodiphenylsulfone (dapsone, DDS), mercurial preparations, herbs and arsenicals in order, were found. The distinct laboratory findings of the disease revealed leukocytosis, eosinophilia and hypoproteinemia. However, the results of liver function tests (SGOT, SGPT, CCF, TTT, Alk. phosphatase) were found to be generally within normal limits in cases of hypoproteinernia. The clinical symptoms of the disease appeared to be lymphadenopathy, coated or avitaminosis type glossitis or stomtitis with characteristic skin lesions of the disease. In the treatment of the disease, the authors emphasized careful treatment to correct fluid and electrolyte balance and hypoproteinemia with adequate topical skin management. The authors also had discussions of the etiological factors comparing with other literatures, clinical signs and symptoms, therapeutical achievement and problems, and reviewed the literatures of the disease.
Age Distribution ; Alanine Transaminase ; Arsenicals ; Avitaminosis ; Child ; Dermatitis, Exfoliative* ; Dermatology ; Eosinophilia ; Glossitis ; Humans ; Hypoproteinemia ; Incidence ; Leukocytosis ; Liver Function Tests ; Lymphatic Diseases ; Skin ; Water-Electrolyte Balance

Twenty-three bacteriologically positive leprosy patients were randomly selected from a leprosarium, randomly divided into four treatment groups, and given vaying dosages of Isoprodian, the tablet contained INAH 175mg, Prothionamide 175mg and DDS 50 mg, for five months. Group I: Initial 1/2 tablet dose per week with 1/2 tablet increment every weeks until 12 tablets were being consumed w.
Humans ; Leprosy* ; Prothionamide ; Tablets