BACKGROUND: Beckers nevus is a benign lesion that may be congental or acquired, macular or papular, hairless or hypertrichotie. OBJECTIVE: Our pcrpose was to describe the clinical findings of Eeckers nevus and to establish a method for the histologic diagnosis of Beckers nevus. MEHTODS: We reviewed PF skin biopsies and their clinical finding from 28 patients with Beckers nevus during a 10 year period from January 1981 to January 1991. Results : The results were summarized as follows. A Clinical characteristics 1. Sex distribution showed of male predominence with male female a ratio of 1.5:1. Age of onset was predominent in less that 5 years old and puberty. 2. The lesions were located on the trunk(24 cases), head & neck(8 cases), extremities(6 cases), and were repsilateral in 26 cases, bilateral in 2 cases. 3. The lesions were accompanied by hair in 17 cases(61%). The shaies of the lesions were reticulated (75 %), patch(18%), mottled(7%). 4. Hairs were distributed as follows : abdomen(100%), scapula(100 %), extremities (83%), cheek(80 %), neck(67 %), chest(40%). All lesions of the face had follicular papules. 5. Associated skin diseases were : smooth muscle hamartoma(3 cases), striae distensa(2 cases), steroid acne(2 cases), pigmented nevus(2 cases), and verruca plana(1 case). B. Histopathologic characteristics 1. The lesions were accompanied with plugs in 21 cases, which were follicular, acrosyringeal, epidermal. 2. Elongation of the rete ridge and hyperpigmented basal cells were observed in the epidermis in all cases. 3. The shape of the rete ridge in Beckers nevus has its characteristic appearance, which is single, bridging and reticulated. Single, bridging and reticulated shapes cexisted in 17 cases(61%). 4. Inflammatiry cell infiltrations were present in all of the upper drmis, in which melanophages(21 %), hair follicles(89%) and smooth muscle hamartoma(3cases) w ere observed CONCLUSION: These findings indicated that the shape of the rete ridge in Beckers nevus has its characteristic appesrance and can be useful for the diagnosis of Beckers nevus.
Adolescent ; Age of Onset ; Biopsy ; Child, Preschool ; Diagnosis ; Epidermis ; Extremities ; Female ; Hair ; Head ; Humans ; Male ; Muscle, Smooth ; Nevus* ; Puberty ; Sex Distribution ; Skin ; Skin Diseases ; Warts

Epidermolysis bullosa simplex (EBS) is a group of autosomal dominantly inherited genetic disorders characterized by blistering due to mechanical- stress-induced degeneration of basal epiderrnal cells. Recently, it was discovered that EBS is induced by keratin 5 and 14 gene mutations. Weber Cockayne (W-C) EBS is the mildest type, with blistering concentrates primarily on palar and plantar regions, and basal cell cytolysis by keratin filament perturbations is present. Herein we report a case of W-C EBS with its ultrastructural findings. Electron microscopy showed cytolysis and separation of the basal epidermal cells, mainly at the subnuclear cytoplasm. The cyto- plasm of basal cells showed edema, loosening and intact rnitochondria. Besides the cytoplasmic changes, the nucleus also showed lytic degeneration. Characteristically, dense condensation of tonofilarnent was observed, which suggests that W-C EBS is. also a disorder of keratin.
Blister ; Cytoplasm ; Edema ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Keratin-5 ; Microscopy, Electron

No abstract available.
Immunoglobulin M*

Hypereosinophilic syndrome is characterized by marked eosinohia and eosinopbilic infiltrition of various organ-systems without any identifiable underlyin sathologic conditions such as hypersensitivity, drug eruption, parasitic infestat,ion, malignant tuinc, and autoimrnune and infectious d iseases. A 31 year-old male had weight loss, abnormal peripheral cirrultiction both extremities and two distinct skin lesions. One showed rice sized, erythematous follicular popules on both extremit.ies and trunk and the other showed a walnut sized, gangrenous ulcer on the left 2nd finger tip. The patients skin lesions and circulation defect. improved after retrnent with steroid and the blood eosinophilia returned to normal.
Adult ; Blood Circulation* ; Drug Hypersensitivity ; Eosinophilia ; Extremities* ; Fingers ; Humans ; Hypereosinophilic Syndrome* ; Juglans ; Male ; Skin ; Ulcer ; Weight Loss

T-cell lymphomas primarily involving subcutaneous tissue and minicking panniculitis have rarely been described. In addition, some cases may have been orviously reported as malignant histiocytosis. Some of the affected patients have developed a hemohagocytic syndrome. A 44-year-old wornan showed multiple, recurrent, ill-defined, sarble sized, erythematous, tender subcutnaeous nodules on the trunk and extremities. Histopathological findings levealed the infiltration of hyperchrorriatiatypical large lymphocytes and nurnerous beanbag-like cells in the subcutaneous fat. The infitrted cells were positively stained with Leucocyte common antigen, UCHL-1 antigen, Lysozyme, that ot with CD20. Bone marrow biopsy showed mild erythreid hyperplasia and erythorophagocytic. hitiocytes. These histopathological features were consistent with cutnaeous T cell lymphoma involvnig the subcutaneous tissue.
Adult ; Biopsy ; Bone Marrow ; Extremities ; Histiocytic Sarcoma ; Humans ; Hyperplasia ; Lymphocytes ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous* ; Muramidase ; Panniculitis ; Subcutaneous Fat ; Subcutaneous Tissue*

PURPOSE: We evaluated the diagnostic accuracy and clinical usefulness of fluoroscopy-guided bone biopsy. MATERIALS AND METHODS: A total of 31 patients with various skeletal lesions underwent fluoroscopy-guided bone biopsies. The targets were long bones in 16 cases, pelvic bones in 7 cases, spines in 6 cases, and ribs in the rest 2 cases. RESULTS: The overall accuracy was 71%(22/31). The accuracy was 100% in case of primary sarcoma and multiple myeloma. But it was low in malignant lymphoma(1/4), malignant fibrous histiocytoma(0/1), and nonossifying fibroma(0/1). CONCLUSION: Percutaneous fluoroscopy-guided bone biopsy is a simple, safe, not-expensive, and rather accurate diagnostic procedure in evaluation of bone tumors. In addition to that, it is useful in the managment planning and fllow-up of the patients.
Biopsy* ; Humans ; Multiple Myeloma ; Pelvic Bones ; Ribs ; Sarcoma ; Spine

The basic pathophysiology of vitiligo is still obscure. Most researchers emphasized that poasible immunologic role is very important in pathophysiology of vitiligo. Also the type of vitiligo is classified by various type baaed on clinical manifestations. These facts promoted us to analyse the immunologic state in each type of vitiligo in order to verify whether there is present any immunologic alteration in this permatosis or any differences of immune state in each type of vitiligo. The following immune cells were analysed, T cell, B cell, and T cell subsets such as helper T cell and suppresaor T cell. Vitiligo vulgaris in our study showed alteration of immune cell such as low level of T cell and helper T cell.
B-Lymphocytes* ; T-Lymphocyte Subsets* ; Vitiligo*

No abstract available.
Meningitis, Bacterial* ; Stapes*

We reviewed the clinical features, histrologic patterns and clinical courses of 30 children with lupus nephritis retrospectively, and the results were summerized as follows; 1) The male to female ratio was 1:2.8, and the mean age at the onset was 10 8/12 years. 2) The clinical symptoms were diverse, and malaise, weight loss, anorexia, fever and malar rash were the most frequent findings. 3) Among the immunologic tests, FANA and anti-ds-DNA test revealed the highest sensitivity with positive rates of 97% and 87%, respectively. 4) Clinically, 57% of patients had active nephrotic syndrome at the onset, and 33% showed (?) was the most common findings (70%). 5) During the follow-up period, one children with Class IV lupus nephritis expired. And 8 out of 9 cases with renal insufficiency at the onset showed improvement of renal function after treatment with corticosteroid and cytotoxic agents. In conclusion, the clinical features and histologic findings of lupus nephritis in children were diverse. Early diagnosis and proper treatment can prevent rapid deterioration of renal function and improve long-term survival rate.
Anorexia ; Child* ; Cytotoxins ; Early Diagnosis ; Exanthema ; Female ; Fever ; Follow-Up Studies ; Humans ; Immunologic Tests ; Lupus Nephritis* ; Male ; Nephrotic Syndrome ; Renal Insufficiency ; Retrospective Studies ; Survival Rate ; Weight Loss

Abrupt increase in the size of cervico-mediastinal tumor due to infection or spontaneous hemorrhage into cyst can induce severe tracheal compression and therefore sudden death. A 5 year old boy, who had a history of URI, had an enlarging cystic hygroma on the right side of the neck and anterior mediastinum. Under diagnosis of the cervico-mediastinal cystic hygroma, surgical removal was scheduled. After induction of anesthesia, intubation was done without any difficulty. A few minutes later, signs of partial airway obstruction were appeared. And within a very short period, total airway occlusion occurred. The tracheal tube was removed and manual ventilation was performed with positive airway pressure, but ineffective. We attempted to puncture cricothyroid membrane with 14 Gauge needle in order to ventilate manually. As soon as we puncture cricothyroid membrane, straw-colored fluid, not air, gushed out through a needle. After aspiration of about 200ml of cystic fluid, the obstructive signs disappeared and the patency of the airway was maintained. Intraoperatively, no more airway problems occured and vital signs were stable. And postoperatively, patient had no specific complications and discharged on the 7th day after operation.
Airway Obstruction* ; Anesthesia ; Child, Preschool ; Death, Sudden ; Diagnosis ; Hemorrhage ; Humans ; Intubation ; Intubation, Intratracheal* ; Lymphangioma, Cystic* ; Male ; Mediastinum ; Membranes ; Neck ; Needles ; Punctures ; Ventilation ; Vital Signs