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

Melanocytes were identified by the dopa reaction, and counted in vertical sections of 183 specimens of normal skin and 218 specimens of patients with pigmentary disorders. The incidence of dopa-positive cells in the basal layer was determined. One cell in 9 in the basal layer was dopa-positive in normal specimens. There was no significant difference in the incidence of dopa-positive cells between male and female and between adult and children. Specimens from face, neck and prepuce revealed comparatively high incidence of dopa-positive cells. Among pigmentary disorders, vitiligo and halo area of halo nevus were characterized by an abscence or marked reduction in the number of dopa-positive cells, but lentigo and cafe-au-lait spot were characterized by an increase in their number In tuberous sclerosis, tinea versicolor and idiopathic guttate hypomelanosis, there wasn't any change of the melanocyte population between lesional skin and normal skin. The significance of these findings in vertical skin sections was discussed.
Adult ; Cafe-au-Lait Spots ; Child ; Dihydroxyphenylalanine* ; Female ; Humans ; Hypopigmentation ; Incidence ; Lentigo ; Male ; Melanocytes ; Neck ; Nevus, Halo ; Skin* ; Tinea Versicolor ; Tuberous Sclerosis ; Vitiligo

It is well known that leprosy bacilli have a special affinity for peripheral nerves, especially Schwann cells but also including perineurial cells and endothelial cells of endoneurial blood vessels. The Schwann cells, after invasion by and saturation with multiplied M. leprae, are destroyed and segmental demyelination and Wallerian degeneration follow. This condition, then is known to be the main neural pathogenosis in early leprosy. It is rarely observed that the progressive paralysis occurs in arrested leprosy patients under adequate treatment. Therefore, the authors performed a histopathologic study of 80 peripheral nerves obtained from the autopsies of 10 old lepromatous leprosy patients including two active cases whose disease durations ranged from 6 to 49 years. The authors arbitrarily divided all the histopathologic findings into 5 grades on the basis of 1) difference in pathological findings in relation to disease duration and bacterial index, and 2) the coexistence of various lesions within the same nerve trunk. As the disease progresses the destroyed nerve fibers are replaced by fibrous and granulation tissue while the perineurial reaction increases. The cellular reaction is minimal in the low grades. There is a slightly early increased vasculature probably due to M. leprae and later a more pronounced increase with large vessels indicating the presence of the granulation process. There is minimum to moderate interfascicular reaction throughout every grade, ie. increased cellularity, vessel wall thickening and increased numbers of vessels with fibrosis. Acid-fast bacilli were demonstrated only in the active cases. The coexistence of different fascicular lesions within the same nerve trunk of peripheral nerve and of femoral nerves mear the spinal cord indicates the presence of some perineurial inhibitory effect which, while promoting the centripetal spread of, prevents the transfascicular spread of M. leprae for some time.
Autopsy ; Blood Vessels ; Demyelinating Diseases ; Endothelial Cells ; Femoral Nerve ; Fibrosis ; Granulation Tissue ; Humans ; Leprosy* ; Leprosy, Lepromatous ; Nerve Fibers ; Paralysis ; Peripheral Nerves* ; Schwann Cells ; Spinal Cord ; Wallerian Degeneration

A case of Toxic Epidermal Necrolysis affecting 24 years old, male Korean, who has been suffering from Pulmonary Tuberculosis, is presented. The skin lesions of the case have been developed about two months after administration of Thioacetazone, 150 mg. per day. The cause of this case may be due to Thioacetazone, one of the antituberculous drugs. Diagnosis of Toxic Epidermal Necrolysis was estsblished by clinical characteristics, histopathological and laboratory studies, and review of literatures were made also.
Diagnosis ; Humans ; Male ; Skin ; Stevens-Johnson Syndrome* ; Thioacetazone ; Tuberculosis, Pulmonary ; Young Adult

In order to evaluate the effectiveness of clocortolone pivalate(Purantix) on corticosteroid responsive dermatoses, a symmetical paired. comparison technique was performed using 0. 1% clocortolone pivalate and 0. 025% triamcinolone acetonide creams. During the pericd of 7 months from September 1977 to March 1978, 37 patients with bilateral lesions of contact dermatitis, seborrheic dermatitis atopic dermatitis, lichen simplex chronicus and psoriasis were selected and treated with afore mentioned drugs, In the 15 psoriatic patients, clocrtolone pivalate was superior in 3, triamcinolone acetonide was superior in 2, 4oth drugs were equally effective in 10. In the evaluation of all 37 patients clocortolone pivalate was superior in 8, triamcinolone acetonide was sureior in 3, both drugs were equally effective in 26. The overall superiority of clocortolone pivalate to triamcinolone acetonide was non significant, There was no side effects with either drug. The simplicity of the vasoconstriction tcst has led to its general use in the study of the potency of new topical steroids. In the 6h occluded single-application vasocon-strictor test performed on 40 healthy students, there was no significant difference between clocortolone pivalate and triamcinolone acetonide, but they significantly d.ifferent from 1% hydrocortisone and the control; a simple ointment base(p<0.01).
Dermatitis, Atopic ; Dermatitis, Contact ; Dermatitis, Seborrheic ; Humans ; Hydrocortisone ; Neurodermatitis ; Psoriasis ; Skin Diseases* ; Skin* ; Steroids ; Triamcinolone Acetonide ; Vasoconstriction