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

A case of verrucous hemangiorna appearing 22-year old male as presented, The clinical lesion was wart-like and hyperkeratotic scaly plaque with satellite lesions occurring over the right side of the skin. Histopathological examination showed hyperkeratosis, papillomatosis, irregular acanthosis, and capillary hemangiomatous features in the dermis and subecutantous tissues.
Capillaries ; Dermis ; Hemangioma* ; Humans ; Male ; Papilloma ; Skin ; Young Adult

The authors studied thirty-three cases of malignant tumors of the skin (24 squamous cell carcinomas, 6 basal cell carcinomas, 2 Paget's diseases and one malignant melanoma) at the department of dermatology, Pusan national university hospital during the period from 1966 to 1971. The results obtained were summarized as follows: 1) There were 21 men(63.6%) and 12 women(36.3%) in this series. 2) The average age of these patients was 56 years with a range of 33 years to 70 years. 3) The ages at onset ranged from 29 to 66 years with the peak incidence in the 50-59 years group. 4) Of the 33 cases, 28 cases were first diagnosed at the age of over 40 years. 5) The ratio of squamous cell carcinoma versus basal cell carcinoma was 4: l. 6) In 29 patients the tumors occurred on the exposed areas of the body and the most common predilection site was on the face (63.6%), of which 6 squamous cell carcinomas occurred on the upper lip. 7) Of the 30 cases of both squamous cell carcinomas and basal cell carcinomas, 20 cases (66.6%) showed ulcerative lesions measuring from less than 1cm to 10cm in diameter. 8) Two cases of the tumors (one squamous cell carcinoma and one malignant melanoma) showed metastasis at the time of diagnosis. 9) The accuracy of clinical diagnosis in this series was 96%. 10) Of the 24 cases of squamous cell carcinomas, 7 cases were treated with radiotherapy, 5 cases with curettage and electrodesiccation followed by radiotherapy. Four squamous cell carcinomas and two basal cell carcinomas that showed relatively superficial and small lesions measuring less than 1.5cm in diameter were treated with electrodesiccation and curettage and are under observation with favorable results for six months to two years.
Busan ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Curettage ; Dermatology ; Diagnosis ; Humans ; Incidence ; Lip ; Neoplasm Metastasis ; Radiotherapy ; Skin* ; Ulcer

We reported a case of epidermodysplasia verruciformis which occurred in a 15 year-old girl. She had an extensive eruption of plane warts on the face, neck, upper trunk, and extremities, especially on the dorsa of the hands and feet, with symmetrical diatribution. The skin lesions appeared initially on the forehead and gradually generalized. all over the body for 10 years duration. Skin biopsy showed hyperkeratosis with basket-weave pattern, thickened granular layer, vacuolization of many celIs in the upper stratum malpighii and the granular layer and acanthosis.
Adolescent ; Biopsy ; Epidermodysplasia Verruciformis* ; Extremities ; Female ; Foot ; Forehead ; Hand ; Humans ; Neck ; Skin ; Warts

Pyoderma gangrenosum, as described by Brunsting and his aaaociates in 1930, is characterized by the rapid appearance of one or more bluish-black, boggy, undermined ulcers, most frequently on the legs of peraons with ulcerative colitis or rheumatoid arthritis. Its pathogenesis is often obscure. Present management of pyoderma gangrenosum consists of local treatment of the wound, systemic cortieosteroid therapy, and treatment of any underlying disease. In our 21 year-old male with pyoderma gangrenosum, hyperbaric oxygen therapy was performed. Treatment was given in the hyperbaric oxygen one-man chamber at 3.0 ATA for 30 minutes daily for 49 days. By 36 days, the ulcers on the face and neck was healed. Approximately one month after the patient was discharged, complete resolution of the 1esions on the back occurred.
Arthritis, Rheumatoid ; Colitis, Ulcerative ; Humans ; Hyperbaric Oxygenation* ; Leg ; Male ; Neck ; Oxygen ; Pyoderma Gangrenosum* ; Pyoderma* ; Ulcer ; Wounds and Injuries ; Young Adult

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*

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