A case of Vogt-Koyanagi-Harada's Sindrome chiefly affected the anterior uvea has been clinically studied with several references. It was a typical one with hearing disturbances and was complicated with a secondary glaucoma. Following the iridectomy the I.O.P. was back to normal limit and with steroid therapy the inflammatory signs were relieved. After a considerabie period the anterior uveitis recurred. In this case the age of the patient was 10 years older than those Parker presented.
Child ; Glaucoma ; Hearing ; Humans ; Iridectomy ; Uvea ; Uveitis, Anterior

The alterations in the localization of keratinocyte membrane glycoconjugates in allergic contact dermatitis were investigated in guinea pig skin treated with topical application of 2.4-dinitro-chlorobenzene. We employed the avidin-biotin complex(ABC) method for the detection of localization of 10 commercially available lectins labelled with biotin: Con-A, SBA, WGA, DBA, UEA-1, RCA-1, PNA, HP, MPA, and ECA. Staining with WGA showed a remarkably decreased intensity in basal and spinous layers of the allergic skin in comparison to those of the control skin, suggesting loss of terminal sialic acids in cell membrane glycoconjugates. The other lectins showed no remarkable difference in the staining patterns between the normal and the allergic ski. The results suggest that epidermal cell membrane glycoconjugates undergoes selective perturbations in acute allergic contact dermatitis, and that the keratinocytes might be an active part of the cutaneous immune system.

Rhinoscleroma, or scleroma, is a chronic, slowly progressing granulomatous disease involving the upper respiratory tract, especially the nasal vestibules, choanae, pharynx, and larynx. Almost certainly the causative agent is Klebsiella rhinoscleromatis. The disease occurs frequently in Eastern Europe, the Middle East, and parts of Latin America, but it is hard to find such case in Korea. We prisent a case of rhinoscleroma involving the nasal vestibule in a 18-year-old male who was admitted due to nasal obstruction for 5 years and epistaxis for 2 months. The resected specimen was an irregular polypoid mass with relatively firm consistency and measured 3 cm in the largest diameter. Microscopically, the lesion was characterized by extensive fibrosis and inflammatory cell infiltration. The infiltrates consisted of predominantly lumphocytes, plasma cells, foamy or granular histiocytes which were singly scattered or grouped in clusters. In the cytoplasm of the histiocytes, round slightly basophilic bodies were noted. Warthin-Starry satin showed short positive rods within the cytoplasm of the cell (Mikulicz cell). Electron microscopically, the cytoplasm of Mikulicz cells contained large, round or irregular shaped clear vacuoles in which numerous Klebsiella bacilli attached to the boundaries of the vaculoes were noted. With higher magnifications, the bacilli were seen as roung or rod-shaped organisms.
Male ; Humans

Cobb syndrome is a rare neurocutaneous angiomatosis characterized by a vascular skin nevus associated with a spinal cord angioma of the same metamere. A 14-year-old girl had an asymptomatic large cutaneous hemangioma distributed from the TI dermatome downward to the L3 dermatome since birth and complained of a gait disturbance and urination difficulty for 1 year. A biopsy specimen in the skin lesion revealed the findings of capillary hemangioma. From C7 downward to L4 posterior epidural hemangioma composed of arteriovenous and cavernous components was diagnosed by radiological examination and surgical exploration. Because of very extensive cord hemangioma, only partial removal of the tumor at T11, T12 and L1 level was performed and postoperatively she was transferred to a special facility for rehabilitative therapy.
Adolescent ; Angiomatosis ; Biopsy ; Female ; Gait ; Hemangioma ; Hemangioma, Capillary ; Humans ; Nevus ; Parturition ; Skin ; Spinal Cord ; Urination

No abstract available.
Hantaan virus* ; Hemorrhagic Fever with Renal Syndrome*

Fabry's disease is a rare hereditary metabolic disease caused by alpha-galactosidase deficiency, resulting in abnormal accumulation of galactosyl galactosyl galactosyl ceramide (ceramide trihexoside) in various organs. Articles in English reported approximately one hundred cases but only two cases in Korea. Recently the authors experienced a case of Fabry's disease of a male patient and studied the electronmicroscopy on skin biopsied tissue and enzyme assay of alpha -galactosidase activity in his peripheral blood leukocytes. The male patient was a 21-year-old soldier who suffered from anhidrosis with heat intolerance and generalized telangietatic papules (Angiokeratoma corporis diffusum) since childhood. Other clinical findings were ocular change, paroxysmal pain of lower extremities and proteinuria with oval fat bodies on urinalysis. The ultrastructural study of skin demonstrated abnormal lysosomal deposits of finger-prints or "zebra" body configuration in the endothelial cells, pericytes, perineural cells and intercalated ductal epithelium of sweat glands. Enzyme activity of alpha-galactosidase was markedly decreased in the peripheral blood leukocytes comparing to the normal control, which was conclusive to make a diagnosis of Fabry's disease.
Child ; Male ; Female ; Humans

Fabry's disease is a rare hereditary metabolic disease caused by alpha-galactosidase deficiency, resulting in abnormal accumulation of galactosyl galactosyl galactosyl ceramide (ceramide trihexoside) in various organs. Articles in English reported approximately one hundred cases but only two cases in Korea. Recently the authors experienced a case of Fabry's disease of a male patient and studied the electronmicroscopy on skin biopsied tissue and enzyme assay of alpha -galactosidase activity in his peripheral blood leukocytes. The male patient was a 21-year-old soldier who suffered from anhidrosis with heat intolerance and generalized telangietatic papules (Angiokeratoma corporis diffusum) since childhood. Other clinical findings were ocular change, paroxysmal pain of lower extremities and proteinuria with oval fat bodies on urinalysis. The ultrastructural study of skin demonstrated abnormal lysosomal deposits of finger-prints or "zebra" body configuration in the endothelial cells, pericytes, perineural cells and intercalated ductal epithelium of sweat glands. Enzyme activity of alpha-galactosidase was markedly decreased in the peripheral blood leukocytes comparing to the normal control, which was conclusive to make a diagnosis of Fabry's disease.
Child ; Male ; Female ; Humans

The pubic louse, Pthirus pubis is a blood-sucking ectoparasite adapted to hold onto pubic, axillary and body hairs. It is usually confined to the pubic and inguinal region. However, it may also rarely involve the scalp and eyelashes. We report two cases of phthiriasis occurring on unusual sites, the first case occurring on the scalp of a 5 month-old male infant, and the second case on the eyelashes of a 49-year-old fe-male.
Eyelashes ; Hair ; Humans ; Infant ; Male ; Middle Aged ; Phthiraptera ; Scalp

In order to investigate the distribution patterns of the dorsal digital nerves of the radial and ulnar nerve in the Korean, authors dissect the 113 hands (right 58/left 55) of the 59 cadavers (39 males/20 females). The types were classified by the area of radial dorsal digital nerves and the ulnar digital nerves. The difference in the distribution pattern between males and females, right and left hands was analysed by chi2-test in the case presenting the prequency more than 10%. The results as follows; 1. The ten types of the distribution patterns consisted of the radial and ulnar nerves were observed on the dorsum of the hands. 2. The case of the highest prequency was type VIII(33.9%), in which radial nerve supply the radial side of the 2 1-2 of digits and ulnar nerve extends the ulnar side 2 1-2 of digits. 3. In the cases of the both nerve mingling in the third digital web, the incidences in which the radial nerves extend to the radial half of ring finger and ulnar nerve to the ulnar half of middle finger (type III) were 25.7%, and that the radial nerves extend to the ulnar half of middle finger and ulnar nerve to the ulnar half of middle finger (type VI) were 11.0%. 4. Type IV as combined branch between the radial and the ulnar nerve extend to the third digital web was observed in the 12.8%. 5. The type III, VI, IX, X, XI showing the both nerves mingling in the third digital web and in the second digital web or combining in the second digital web were new observed in the Korean. 6. The musculocutaneous nerve replaces the superficial branch of the radial nerve in 4 cases. 7. There was no statistical difference in the distribution pattern between males and females, right and left hands. From the above results, it was suggested that the majority of the cases were that the ulnar digital nerves supplied the ulnar half of the middle finger in the Korean.
Cadaver ; Female ; Fingers ; Hand* ; Humans ; Incidence ; Male ; Musculocutaneous Nerve ; Radial Nerve ; Ulnar Nerve

The Sturge Weber syndrome is an uncommon disorder characterized by port-wine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated with this syndrome are mental retardation, choroidal angioma, buphthalmus or glaucoma, seizure and hemiplegia. We studied a case of incomplete form of Sturage Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calcification and leptomeningeal angiomatosis. There are no characteristic facial nevus, mental retardation, occular changes and hemiplegia.