Nineteen years old female with typical pseudoxanthoma elasticum without family history of the disease, and 26 years old male with characteristic clinical and histopathologic findings of Groenblad-Strandberg syndrome and family history of pseudoxanthoma elasticum in his 9 years old sister were presented.
Adult ; Child ; Female ; Humans ; Male ; Pseudoxanthoma Elasticum* ; Siblings

Enterotoxigenic B. pagilis (ETBF) strains which produce a 20 kDa zinc metalloprotease toxin (BFI) have been associated with diarrheal disease of animals and young children. Using B. pngilis toxin gene (bfi) from strain 86-5443-2-2 (piglet isolate) as a probe, the gene was identified in 74/77 human and animal ETBF strains but only 2/97 non-toxigenic B. fragilis (NTBF) strains. The region flanking bp was mapped with several restriction enzymes and 8 resriction fragments aacent to bft were used to probe colony blots of 77 KTBF and 97 NTBF strains. All 74 bft-positive ETBF strains hybridized to the 8 probes spanning a ca. 18 kb chromosomal region; however, this 18 kb region was absent in the 3 ETBF strains lacking p, and 47 of the 97 (48%) NTBF strains lacked the entire 18 kb region. Of note, the 2 NTBF strains containing btf did not have a ca. 12 kb region upstream of btfp. A ca. 9 kb fragment flanking the btf gene has been sequenced. Analysis of this data revealed several open reading frames (ORF) of which 3 are of particular interest (ORFs 1, 2 and 3). ORF1 and ORF3 encode proteins with significant homology to mobilization proteins, and ORF2 encodes a protein with significant homology to metalloprotease proteins, but only 50% similarity and 30% identity to BFf. These results suggest: 1) the btf genes are flanked by at least 18 kb of DNA largely unique to ETBF strains indicating a putative pathogenic island, 2) another metalloprotease protein present in ETBF strains may contribute to the pathogenicity and variable virulence of these diarrheagenic strains and 3) the pathogenic island may be mobiTized among different Bacteroides strains, and possibly among different species of intestinal bacteria.
Animals ; Bacteria ; Bacteroides fragilis* ; Bacteroides* ; Child ; DNA ; Genomic Islands* ; Humans ; Open Reading Frames ; Virulence* ; Zinc

This study was performed with 31 cases of cutaneous tuberculosis among 18,846 cases of out-patients in Dermatologic Department of Busan National Universicy Hospital from 1967 to 1976. These 31 cases were consistent with skin tuberculosis in histological and clinical findings. The results were as follows. 1. The incidence of skin tuberculosis is 0.16%, among 18,846 cases of outpatients, 2. The localized tuberculosis of the skin, i.e. lupes vulgaris, tuberculosis verrucosa cutia and scrofuloderma was more common than disseminated type of tuberculosis of the skin. Among the disseminated types, papulonecrotic tuberculid is rather common than other types. 3. The ratio between male and female was l. 2: 1. 4. The age of onset varied according to types, but 70% of all cases occurred in the age group between 10-30 years. 5. Lung tuberculosis was associated in abaut 20 of skin tuberculosis. 6. Tubercle and tuberculoid structure were noticed in all biopsy specimens ancl in 55% the caseation necrosis was noticed.
Age of Onset ; Biopsy ; Busan ; Female ; Humans ; Incidence ; Lung ; Male ; Necrosis ; Outpatients ; Skin* ; Tuberculosis ; Tuberculosis, Cutaneous*

Familial Renign Chronic Femphigus(FRCP) is rare, persistent and vesicobullous genodermatoai. Three casee of FRCP with typical clinical and histapathological findings are reported. The first case of FBCP was 27-year-oId man who had recurrent episodes of grou vesicles and rust with miId pruritue at the posterior neck, in both axillae and in inguinal area for 10 years. The second case was 49-year-old woman with skin lesions of recurrent persistent. vesicles, pustules on the erythematous skin, crust and lichenification on the flexural area of extrimeties, axillae, ingumal and neck area for 10 yeara. The third case was 49-year-old male patient who had recurrent pruritic grouped vesicles with crust on the neck and axillary area. In all three patients, skin eruptions usually began and or aggravated in the spring or summer and tend to heal spontaneously in the autumn and winter and showed positive Nikolsky sign. The only one case of the reported three casea had familiaI history of skin lesions similar to FRCP. Histopat,holag;ic finding of biopsy specimens from the vesicular eruption showed auprabasal vesiculation with acantholysis, lacunae and villi formation in all 3 cases.
Acantholysis ; Axilla ; Biopsy ; Female ; Humans ; Male ; Middle Aged ; Neck ; Pemphigus, Benign Familial* ; Skin

Nevus Lipomatous cutaneous superficialis (Hoffman-Zurhelle) is distinct clinical entity. A case is reported in which the lesions appeared in childhood and were elevated plaques and nodules in distribution on the tight side of the buttock.
Buttocks ; Nevus*

We reported a case of follicular mucinosis which occurred in a 51 year old m;', . He had a firm grouped folliculopapular plaque above left eyebrow with ill-defined border, without any loss of eyebrow. But histopathologic findings showed charac- teristic changes in hair follicles and sebaceous glands with mucinous degeneration and no evidence of suggesting mycosis fungoides. Relatively rapid response to systemic corticosteroid suggested that this therapy had an actual effect.
Eyebrows ; Hair Follicle ; Humans ; Middle Aged ; Mucinosis, Follicular ; Mucins ; Mycosis Fungoides ; Sebaceous Glands

Clinical studies on five patients with acrodermatitis enteropathica visited during the period from March 1968 to September 1970 to the department of dermatology, Pusan national University hospital were made and the results obtained were summerized as follows; in addition, the literature was reviewed. 1) All of 5 patients aging from 3 months to 3 years, showed characteristic distributions of the cutaneous lesions which ranged in character from vesicobullous to heavily scaled psoriasiform and moniliasis-like lesions. 2) Of these, the nearly full clinical pictures were presented in two cases with the gradual onset in early infancy, dermatitis predominantly involving the periorficial areas and extremities, which followed by recurrent attacks of greenish yellow-colored diarrhea, partial and diffuse loss of the scalp hairs, stomatitis and monilial infections, whereas the rest thtee cases seemed to be "forme fruste" of this disease with the absence of hair and nail abnormalities. 3) There found no speeific histologic findings in two biopsies performed but showed somewhat the pictures of sub-acute dermatoses. 4) Candida albicans were demonstrated from the skin lesions in two cases, 5) Treatment with local application of gentian violet solution, nystatin ointment for the skin lesions and stomatitis were temporarily favorable in all cases and in case 1 and 5, diodoquin, each 300mg and 600mg were administered orally in divided doses for two weeks, with the result of satisfactory responses. However, the latter died, on the 3rd hospitalized day, of acute glomerulonephritis and upper respiratory infection. 6) Though it is generally accepted that there is definite familial occurrence in this disorder but we recognized no evidence of familial incidence in our five cases.
Acrodermatitis* ; Aging ; Biopsy ; Busan ; Candida albicans ; Dermatitis ; Dermatology ; Diarrhea ; Extremities ; Gentian Violet ; Glomerulonephritis ; Hair ; Humans ; Incidence ; Iodoquinol ; Nails, Malformed ; Nystatin ; Scalp ; Skin ; Skin Diseases ; Stomatitis

Two cases of toxic epidermal necrolysis (Lyell) appearing on 69-year old male and 12year-old female were presented. The clinical manifestations of toxic epidermal necrolysis developed after oral administration of novaquing for common cold. The cause of the disease was probably due to novaquing (phenobarbital and sulpyrin). The 69 year-old male was expired despite intensive treatment with fluid and electrolytes, antibiotics, corticosteroids, vitamins, and topical measures, but 12 year-old female was cured successfully with treatment.
Administration, Oral ; Adrenal Cortex Hormones ; Aged ; Anti-Bacterial Agents ; Child ; Common Cold ; Electrolytes ; Female ; Humans ; Male ; Stevens-Johnson Syndrome* ; Vitamins

A patient with keratoma hereditaria mutians, 20 years old Korean girl, presented the following classic features of this rare disease, palmar and plantar hyperkeratosis beginning early in life, starfishlike keratosis of the dorsa of the hands, hyperkeratotic patches of dorsa of the feet with thickening of the toe nails, keloid-like keratoma of the elbows and knees, and constriction of the all fingers begining in early life and spontaneous amputation of theleft 5th finger. No family history was demonstrated in this patient.
Amputation ; Constriction ; Elbow ; Female ; Fingers ; Foot ; Hand ; Humans ; Keratosis* ; Knee ; Rare Diseases ; Toes ; Young Adult

A clinical study was done on 43 patients witb basal cell carcinoma encountered in the Department of Dermatology, Busan National University Hospital during the past 10-years period frorn January 1968 to December 1977. Tbe results were summarized as follows: 1) Of 43 patients, 15 ceses were male and 28 cases were female with ratio of 1: 1.87. 2) Age incidence ranged frorn 15 years to 87 years with the highest incidence rate in the age group 40-49 years and incidence rate increased remarkbly in numlxr after over 40 years of age. Average age was 55 years. 3) The most common site was face occuring in 39 casts(90.7%), comprising 18 cases on nose, 12 cases on eyelids, 6 cases on cheeks, each 1 case on forehead, upper lip and auricle. 4) Noduloulc rative lesion was most common, otscrved in 29 cases(67.4%), nodular lesion in 9 cascs(20.9%), and pigmented lesion in 5 csses(11.6%). 5. The ratio of basal cell carcinoma was 1:1.2. 6) Three cases of precancerous states including 2 cases of xeroderma pigmentosa and 1 case of irradiated skin lesion were observed.
Busan ; Carcinoma, Basal Cell ; Cheek ; Dermatology ; Eyelids ; Female ; Forehead ; Humans ; Ichthyosis ; Incidence ; Lip ; Male ; Nose ; Skin