A case of porphyria cutanca tarda is reported, in which the photosensitive cutaneous symptoms developed in association with impaired hepatic function possibly related to chronic consumption of alcohol. Wood's light examination of the urine revealed coral red fluorescence, and qualitative chemical urinalysis was strong positive for uroporphyrin and coproporphyrin. Biopsy of the skin showed subepidermal bulla with festooning arrangement of the naked papillae and basophilic degeneration of the dermis. The patient's favorable response to alkalinization therapy by sodium bicarbonate per os is encouraging, even for a short period's observation. Clinical, histopathological, and biochemical features of this disease are also discussed.
Anthozoa ; Basophils ; Biopsy ; Dermis ; Fluorescence ; Porphyrias ; Skin ; Sodium Bicarbonate ; Urinalysis

We have presented three cases of Ritter's disease appearing on 11, 14, and 14 days old boys respectively. Staphyloccccus albus was cultured from bullar contents of case 1 and 2, and coaguIase positive staphylococcus from case 3. These patients were successfully treated with systemic and topical antimicrobials.
Humans ; Staphylococcal Scalded Skin Syndrome* ; Staphylococcus

No abstract available.
Neurofibromatoses*

Multiple neurilerv,omas occurred i.n a 14-year-old boy with neurofibromatosis and right eye blindness. His father succumbed to neurofibromatosis at the age of 42. Histologically-proved neurilemornas and neurofibromas were noted on the face, neck, trunk and extremities, but no cafe au-Iait spots ohserved. There were nevus anemicus, hairy nevus and pigmented moles. The associated features in this patient includc muscular atrophies, weakness of the left hand, parest,hesia of the medial side of left forcarm and Dupuytrens contracture of left fourth finger.
Adolescent ; Blindness ; Dupuytren Contracture ; Extremities ; Fathers ; Fingers ; Hand ; Humans ; Male ; Muscular Atrophy ; Neck ; Neurilemmoma* ; Neurofibroma ; Neurofibromatoses* ; Nevus ; Nevus, Pigmented

A case of pachyonychia congcnita occurring in a 8 year-old boy is described. A few weeks after delivery, his mother noticed thickening of all the finger and toe nails of her baby. Family history was denied. In addition to characteristic features of pachyonychia congenita of all the nails, follicular keratotic papules were noted on the knees, elbows, buttocks and legs, and also tender thick calluses, on the soles with palnioplantar hyperhidrosis. There were three cornu cutaneurn on the popliteal and left antecubital fossae. Other associated fea.tures include a painful tense bulla at the left fourth finger, irnpetiginous patches on the anterior thighs, hoarseness and leukokeratosis linguae, sparseness of the scalp hairs, and granulosis rubra nasi accompanied by facial hyperhidrosis.
Bony Callus ; Buttocks ; Child ; Elbow ; Fingers ; Hair ; Hoarseness ; Humans ; Hyperhidrosis ; Knee ; Leg ; Leukoplakia ; Male ; Mothers ; Nails, Malformed* ; Pachyonychia Congenita* ; Scalp ; Thigh ; Toes ; Transcutaneous Electric Nerve Stimulation

Two cases of tuberous sclerosis are reported. Case 1: A 13 years-old girl had a triad of adenoma sebaceum, mental deficiency and epileptic seizures associated with ichthyosis vulgaris, but family history revealed no evidences of the disease except ichthyosis in her mother. Case 2 : A 21 year-old woman had a adenoma sebaceum, shagreen patch, periungual fibroma and fihromatous plaque, showing intracranial calcificatins on roentgenographic examination, but has not demonstrated any neurological manifestations as yet. Family history also revealed adenoma sebaceum in her father and two younger sisters. We feel this case is a "forme fruste" of tuberous sclerosis.
Adolescent ; Epilepsy ; Fathers ; Female ; Fibroma ; Humans ; Ichthyosis ; Ichthyosis Vulgaris ; Intellectual Disability ; Korea* ; Mothers ; Neurologic Manifestations ; Siblings ; Tuberous Sclerosis* ; Young Adult

A case of dermatitis herpetiformis assaciated with erythema annulare centrifugum is reported. The patient was a 21 year-old male, who had papulovesieles, erythematous lesions, pigmentation, and erythema annulare centrifugum lesions on the trunk, shoulders, buttocks and extensor surfaces of the extremities, and severe itching sensation of 9 months' duration. The skin biopsy from the vesicular and erythema annulare centrifugum lesion showed subepidermal bulla and vesicles with neutrophil and eosinophil infiltrations.
Biopsy ; Buttocks ; Dermatitis Herpetiformis* ; Dermatitis* ; Eosinophils ; Erythema* ; Extremities ; Humans ; Male ; Neutrophils ; Pigmentation ; Pruritus ; Sensation ; Shoulder ; Skin ; Young Adult

Acetabular erosion, intrusion of the prosthesis into the pelvis, is the most serious late complication of hemiarthroplasty. For the detailed observation on this lesion, the authors reviewed 55 patients who underwent femoral head replacement and were followed up to at least 1 year after operation. The acetabular erosion were present in 18 patients(32.7%) out of the total 55 patients. Based on the results of this study, the authors presented a new classification of acetabular eorsion: Stage I(erosion of the cartilage only) was seen in 11 patients, stage II (evidence of the initial bony erosion) in 4 patients,and stage III(intraosseous intrusion of the metallic head) in 3 patients. Revision arthroplasty was obligatory in two of the three stage III patients. The severity of symptoms did not always correspond with the radiological changes. The acetabular erosion was more common in younger patients, in male patients and in patients with avascular necrosis of femoral head than in older patients, in female patients, and in patients with femoral neck fracture. The tendency of acetabular erosion occurring mainly in younger and male patients is probably attributed to their better survival and greater activity.
Acetabulum ; Arthroplasty ; Cartilage ; Classification ; Female ; Femoral Neck Fractures ; Head ; Hemiarthroplasty ; Humans ; Male ; Necrosis ; Pelvis ; Prostheses and Implants

The tibia is the most commonly fractured bone of all the long bones. In this age of vehicular accidents, the tibia is frequently subjected to high energy trauma. Anatomically the tibia has poor soft tissue coverage and poor blood supply. Therefore severe injury on the tibia can lead to severe complication and major disability. 41 adult patients with comminuted fracture of the tibial shaft were treated by OR/IF with D.C.P. and bone graft at the Department of Orthopedic Surgery, Kyungpook University Hospital from January 1978 to June 1986. These were analysed according to mechanism of injury, degree of comminution, timing of operation, soft tissue injury and operation time. Bone union and end results of treatment were reviewed. The results were as follows Of 41 cases, causes of injury were vehicular accident in 32 cases. 34 cases had associated injury on other part of body and average injury per case was 1.7 injuries. Based on the classification of Johner and Wruhs, the B group with butterfly fragment was 27 cases and C group was 14 cases. Among the B group, B2 was most common as 14 cases. Average union time was 18 weeks and delayed union and nonunion were 6 cases. Degree of comminution of fracture might be important fractor to bone union. According to the end result of Johner and Wruhs, excellent and good were 31, fair and poor were 10. Of the 41 cases, 10 cases had permanent disability. 4 cases had angular and rotational deformity alone and 1 case had shortening of tibia more than 1 cm, 1 case had both of them. Limited R.O.M. of ankle joint (>50%) occured in 4 cases. The post-operative infection occured in 6 cases, 3 were superficial infection but 3 cases were osteomyelitis. OR/IF with D.C.P. on comminuted tibial shaft fracture was a risk operation. Therefore proper method of treatment should be selected according to degree of comminution, timing of operation, presence or abscence of open wound and associated injury.
Adult ; Ankle Joint ; Butterflies ; Classification ; Clinical Study ; Congenital Abnormalities ; Fractures, Comminuted ; Gyeongsangbuk-do ; Humans ; Methods ; Orthopedics ; Osteomyelitis ; Soft Tissue Injuries ; Tibia ; Transplants ; Wounds and Injuries

Soft tissue defect on dorsum of hand requires immediate soft tissue cover to achieve early wound closure and to minimize wound infection and scarring. If the tendon or bone was exposed and the deep structures were absent, flap was required to provide adequate protection and allow proper function of the underlying structures. Small size in skin loss can easily be covered with local flaps but major defects involving the dorsal aspect of the hand should be coverd with distant flaps, free flaps or radial forearm flaps. Techniques in distant flaps require staged procedures and free flap transfer to obtain soft tissue cover in a single stage require microsurgical techniques. The reverse radial forearm flap is a fasciocutaneous flap based on the radial artery which together with its two venae comitantes lies invested in a condensation of the deep fascia known as the lateral intermuscular septum. The authors had treated 5 cases of distally based reverse forearm flap for soft tissue reconstruction on 2 patients of crushing injury with soft tissue defect on hand, a patient with Marjolin's ulcer on dorsum of hand due to previous burn scar and a patient with soft tissue necrosis on dorsum of both hands due to extravasation of adriamycin during chemotherapy. It has proved to be one of the saftest, simple and effective one-stage method of soft tissue reconstruction in hand without long term immobilization. The results of these operation in cover of soft tissue defect have been satisfactory.
Burns ; Cicatrix ; Doxorubicin ; Drug Therapy ; Fascia ; Forearm ; Free Tissue Flaps ; Hand ; Humans ; Immobilization ; Methods ; Necrosis ; Radial Artery ; Skin ; Tendons ; Ulcer ; Wound Infection ; Wounds and Injuries