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*

Dyschromatosis Symmetrica Hereditaria (Toyama) is one of the pigment anormaly hereditary skin disease which is known to manifest the Japanese. Various authors such as Toyama, Matsumoto, Komaya-Dohi and Ichikawa-Hiraga have reported the disease under different diagnostic name. Clinical characteristics of the disease are shown to have multiple reticular dark brown spotty macular skin lesion mostly over the face, neck, back of hands, feet, fingers and toes, which are affecting symmetrically, without subjective symptom, and occasionally the pigmented and depigmented macular lesions are intermingled. Among these author's reports to the disease, one postulate those which Matsumoto and Komaya-Dohi's cases were essentially similar type in clinical manifestation and were described as showing spotty pigmented macules intermingling with the depigmented over the areas as those of Toyama's. In this article, a case of Dyschromatosis Symemtrica Hereditaria manifesting 21 years old Korean male, which is similar to Matsumoto's type, is presented. Search for the heredity back ground revealed that the grandfather and mother were affected by this disease, and authors assumed the types of heredity of the disease to be irregular non-sexlinked dominant inheritance. Authors presented also distinctions of the histopathological findings of the disease which are hyperkeratosis, increased melanin granules in stratum mucosum layer and imperfect or immatured granular cells, and discussions of differential diagnosis and review of literatures are made.
Asian Continental Ancestry Group ; Diagnosis, Differential ; Fingers ; Foot ; Hand ; Heredity ; Humans ; Male ; Melanins ; Mothers ; Neck ; Skin ; Skin Diseases ; Toes ; Wills ; Young Adult

The authors performed clincial and mycological studies on 185 outpatients who had clinical evidences of tinea cruris (T. cruris) or tinea corporis (T. corporis) and. who had visited the dermatological clinic of Chonnam University Hospital from April to September in 1978. The incidence of T. cruris was 4. 2%, T. corporis 3. 7%, and concurrent infection of T. cruris and T. corporis l. 1% of the 2526 outpatients who visited our clinic during that time. Of the 185 patients, T. cruris was most prevalent among malea, while T. corporis was evenly distributed between both sexes. The age distribution group for T. cruris and T. corporis varied from several months to the eighth decade, but most of them were in their third decade, at an actively working age. Comparing the incidence of urban and rural areas, patients living in urban areas were affected with T. cruris about two times more than those living in rural area but there was little difference regarding T. corporis. Concurrent infection of T. cruris or T. corporis with other dermatophytoses was noted in 42. 2% of the cases. Fatients with T. cruris and tinea pedis (T. pedis) were most, commonly observed, 30. 8%, with T. cruris and T. corporis 28. I r, T, corporis and T. pedis 19. 2Y., and T. cruris and. T. pedis 12. 8% following in order, In etiological studies of T, cruris Trichophyton rubrum (T. rubrum) was most, frequently the causative organism. But Epidermophyton floccosum and Trichophyton. mentagrophytes (T. mentagrophytes) were also commonly isolated. T. corporis was caused most frequently by T. mentagrophytes and T. rubrum, and Microsporum canis (M. canis) and Trichophyton violaceum (T. violaceum) were also isolated. We cultured fungi from each lesion of the 28 patients with concurrent infections of T. cruris and/or T. corporis with other dermatophytoses, and obtained the same causative strains in all lesions of each patient. We presumed concurrent infection of dermatophytosis to frequently be autoinoculation from one part of the body to another, especially in T. pedis which is often the infectious source of T. cruria and/or T. corporis.
Age Distribution ; Arthrodermataceae* ; Epidermophyton ; Fungi ; Humans ; Incidence ; Jeollanam-do ; Microsporum ; Outpatients ; Tinea ; Tinea Pedis ; Trichophyton

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

Familial Benign Chronlc Pemphigus (Hailey-Hailey disease) is relatively rare, persistent, recurrent vesiculo-bullous and inheritable dermatitis. Forty-nine and twenty four year-old two Korean men,affecting classical form of Hailey-Hailey disease, manifested circinated or configurated form of impetiginous, vesiculo-bullous, crusted and pigmented skin lesions over the axillae, neck, shoulder, lower abdornen, inguinal, inner aspect of upper portion of thigh, genito-crural and popliteal space of the skin, There were seen also actively inflammatory border that spread peripherally producing circinate and configurate patterns, and found satellite lesions resembling cutaneous candidiasis in some areas of the lesion. However, eye lesion or lymphnode swelling was not found. Nikolsky sign was present in both patients. The patients had pruritus, intense or slight, over the lesion. The onset of the disease in the cases was mid-twenty and early twenty, and the durations were over twenty years and three years, respectively, and genetic observation on the families revealed that four persons in the two generations and eight persons in the four generations were affected, respectively, and ratio of male to female affected were 5: 1. Routine laboratory examinations including bacteriological and mycological studies revealed non-specific findings. Histopathologic studies showed acantholytic and individual cells in the intra-epidermal bulla, and lacunae, villi, single basal cell lined papillae appearing at the bottom of the bulla, and so-called "dilapidated brick wall" appearance to be seen at the top of blula. Authors discussed the gentic pattern of Familial Benign Chronic Pemphigus (Hailey-Hailey disease) in the two families and presented two cases of the disease with laboratory examinations, histopathological findings and clinical symptoms of the patients, and confirmed that the disease is transmitted as "irregular autosomal dominant" trait, and stressed the difference in frequency, of the diseae according to sex, I.e., in male were more prevalent than female, and reviewed the related literatures af the disease.
Axilla ; Candidiasis, Cutaneous ; Dermatitis ; Family Characteristics ; Female ; Humans ; Male ; Neck ; Pemphigus ; Pemphigus, Benign Familial* ; Pruritus ; Shoulder ; Skin ; Thigh

Familial Benign Chronlc Pemphigus (Hailey-Hailey disease) is relatively rare, persistent, recurrent vesiculo-bullous and inheritable dermatitis. Forty-nine and twenty four year-old two Korean men,affecting classical form of Hailey-Hailey disease, manifested circinated or configurated form of impetiginous, vesiculo-bullous, crusted and pigmented skin lesions over the axillae, neck, shoulder, lower abdornen, inguinal, inner aspect of upper portion of thigh, genito-crural and popliteal space of the skin, There were seen also actively inflammatory border that spread peripherally producing circinate and configurate patterns, and found satellite lesions resembling cutaneous candidiasis in some areas of the lesion. However, eye lesion or lymphnode swelling was not found. Nikolsky sign was present in both patients. The patients had pruritus, intense or slight, over the lesion. The onset of the disease in the cases was mid-twenty and early twenty, and the durations were over twenty years and three years, respectively, and genetic observation on the families revealed that four persons in the two generations and eight persons in the four generations were affected, respectively, and ratio of male to female affected were 5: 1. Routine laboratory examinations including bacteriological and mycological studies revealed non-specific findings. Histopathologic studies showed acantholytic and individual cells in the intra-epidermal bulla, and lacunae, villi, single basal cell lined papillae appearing at the bottom of the bulla, and so-called "dilapidated brick wall" appearance to be seen at the top of blula. Authors discussed the gentic pattern of Familial Benign Chronic Pemphigus (Hailey-Hailey disease) in the two families and presented two cases of the disease with laboratory examinations, histopathological findings and clinical symptoms of the patients, and confirmed that the disease is transmitted as "irregular autosomal dominant" trait, and stressed the difference in frequency, of the diseae according to sex, I.e., in male were more prevalent than female, and reviewed the related literatures af the disease.
Axilla ; Candidiasis, Cutaneous ; Dermatitis ; Family Characteristics ; Female ; Humans ; Male ; Neck ; Pemphigus ; Pemphigus, Benign Familial* ; Pruritus ; Shoulder ; Skin ; Thigh