Epidermolytic hyperkeratosis, previously called bullous congenital ichthyosiform erythroderma is a dominantly inherited disorcler and usually manifested by blisters at or shortly after birth. We report a case of epidermolytic hyperkeratosis in 9 year old girl vrho developed generalized erythema &: easily eroded vesicles at birth. Later the skin lesions progresed to characteristic thick, grayish-dark brown verruform cais, affecting predominantly flexural and intertriginous areas. Fiistologic findings show typical granular degeneration.
Blister ; Child ; Cytochrome P-450 CYP1A1 ; Erythema ; Female ; Humans ; Hyperkeratosis, Epidermolytic* ; Parturition ; Skin

We measured serum 1gE by using IgE 'Eiken' radioirnrnunoassay Kit and delayed cutaneous hypersensitivity responsiveness by the Multitest CMI Kit (Institut Merieux) that simultaneously administrates 7 standardized recall antigens(tetanus, diphtheria, streptococcus, tuberculin, candida, trichophyton, proteus) and 70% W/V glycerin diluent control by multiple prick puncture. We studied 40 cases comprising 20 patients with atopic dermatitis (AD) and 20 healthy children as control. The results were obtained as follows; 1) The mean serum level of IgE in AD(63.3+/-395.2 1U/ml) was higher than in control(155.6+/-67.1IU/ml) (p<0.0l), but the intensity of reaction (IR) & rate of reaction (RR) in AD (l.34+/-1.06, 0.38+/-0.23) was not significantly decreased than in control (1.70+/-0.87,0.44+/-0.16). 2) 1n both AD & control group, tuberculin showed highest IR R RR. 3) Anergic state was found in only one patient with disseminated cutaneous lesions Sc 4) serum IgE, but rate of hypoergy was significantly increased in AD(65%) than in control (30%) (p<0.05). 4) Serum IgE was not statistically correlated with IR and RR, but IR was statistically correlated with RR (p<0.001, r=0.9504). 5) IgE level in severe AD (generalized skin lesion) was significantly increased than in both mild AD 8c control group (p<0. 001). but IR & RR were not statistically correlated with disease severity.
Candida ; Child ; Dermatitis, Atopic* ; Diphtheria ; Glycerol ; Humans ; Hypersensitivity ; Hypersensitivity, Delayed* ; Immunoglobulin E* ; Punctures ; Skin ; Streptococcus ; Trichophyton ; Tuberculin

A 28-year-old male patient is described who developed multiple erythematous nodules and papules on the face, upper chest, back, upper and lower extrimities, leading to extensive ia.filtration of the skin and lymph nodes. Histology from the involved lymph node and skin nodule revealed widely scattered patchy infiltration of abnormal. hyperchromatic polymorphous histiocytes in the dermis. The patient died at about 8 months after onset of the disease, in spite of antineoplastic treatment.
Adult ; Dermis ; Histiocytes ; Humans ; Lymph Nodes ; Lymphoma* ; Male ; Skin ; Thorax

We messured delayed cutaneous hypersensitivity reactions by Multitest CMI Kit. Total 209 normal adults ranging in age from 17 to 55 year(male 106, female 103) were tested. The results were obtained as follows ; 1. Sum of average diameter of positive antigens wss 20.7mm in male and 15.6mm in female. 2. Number of positive antigens was 3.9 in male and 3.4 in female. 3. Rate of anergy was 1.4%(0.9% in male, 1.9% in female). 4. The rate of subjects below warning score(below 10mm in male, below 5mm in female) was 2.8% in male and 5.8% in female. 5. Antigen with the highest response was tuberculin in both sex(male 95.3%, female 89.3%) and antigen with the lowest response was trichophyton(22.5%). 6. Aversge score of positive response of every antigens was highest in tuberculin at both sex(male 2.8mm, female 2,6mm) 7. Average score of total antigens with positive response was 5.5mm in male and 4.6mm in female. 8. The rate of false positive reaction to 70% W/V glycerine was 1.4% totally.
Adult* ; False Positive Reactions ; Female ; Glycerol ; Humans ; Hypersensitivity* ; Korea* ; Male ; Tuberculin

we report a case of elastosis perforans serpiginosa associated with pseudoxanthoma e1asticurn on t:he neck of 27-year-old male patient. The lesions showecl erythematous scaly hyperkeratotic papules forming serpiginous pattern on yellowish elevated plaques. A skin biopsy specimen from the neck lesion revealed dense accurmulations of coarse elastic fibers partly cngulfed by epithelium in the papillary dcrrnis and increase cif short, irregulary clumped and degenerated clastic fibers in the middermis. On funduscopic exarnination, angioid streak is noted around the optic disk.
Adult ; Angioid Streaks ; Biopsy ; Elastic Tissue ; Epithelium ; Humans ; Male ; Neck ; Optic Disk ; Pseudoxanthoma Elasticum* ; Skin

We report a case of histiocytic cytophagic panniculitis in a 5-year-old male. The patient has complained of multiple erythematous indurated palques with tenderness on the lower abdomen, periocular areas, left forearm and both thighs associated with high fever for 45 days. Hepatosplenomegaly and enlarged lymph nodes were found. Laboratory data revealed pancytopenia, elevation of liver enzymes and prolon gation of PT and PTT. Histopathologic findings of subcutaneous plaque taken from the left forenrm showed lobular panniculitis with fat necrosis and some phagocytic histiocytes containing erythrocytes, leukocytes, lymphocytes and nuclear debris, that had the appearance of bean bag. There found no atypical histiocytic infiltrations or cellular pleomorphism.
Abdomen ; Child, Preschool ; Erythrocytes ; Fat Necrosis ; Fever ; Forearm ; Histiocytes ; Humans ; Leukocytes ; Liver ; Lymph Nodes ; Lymphocytes ; Male ; Pancytopenia ; Panniculitis* ; Thigh

A case of generalized pustular psoriasis in a 12 year-old girl is described. She had generalized pustular eruptions on erythematous base, presenting lake of pus, yellowish brown crusts and denudation associated with high fever and chills. With the administration of oral etretinate at a dose of 0.8mg/kg/day for 1 week, rapid and dramatic remission was achieved. About 3 months after her discharge, there developed another wave of new pustules with fever. She was again placed on etretinate therapy. However, there was no expected response as before with developing new pustules continuously. At that time, we tried combination therapy with etretinate and methotrexate. the pustules and erythema began to remit, with eventual clearing of the skin in 6 weeks.
Acitretin* ; Child ; Chills ; Erythema ; Etretinate* ; Female ; Fever ; Humans ; Lakes ; Methotrexate* ; Psoriasis ; Skin ; Suppuration

No abstract available.

An asymptomatic 26-year-old man was initially admitted with a suspicious mediastinal mass. On the basis of the contrast-enhanced chest CT findings, aneurysm formation involving the left cervical aortic arch associated with subaortic left innominate vein was diagnosed. The aneurysm was confirmed by MR angiography and DSA. The arch aneurysm was surgically removed. We describe this case, and review the literature.
Adult ; Aneurysm* ; Angiography ; Aorta, Thoracic* ; Brachiocephalic Veins* ; Humans ; Tomography, X-Ray Computed

We report a case of 68-year-old male patient who was confirmed as d'emblee type of mycosis fungoides. The skin lesions showed sharply rnargined multiple reddish brown coIarcd papules, ndules, and tumors on the groin, thign, abdomen, and neck. The microscopic findings showed the epidermotropism and pautrier's micruabscess in the epidermis, and the infiltration of atypical cells in the dermis. The electron micrascopic findings showed irregular contour of the nuclear mernbrane within atypical lymphocytes. Thc lesions were gradually diminished by superficial radiation therapy.
Abdomen ; Aged ; Dermis ; Dronabinol ; Epidermis ; Groin ; Humans ; Lymphocytes ; Male ; Mycosis Fungoides* ; Neck ; Skin