No abstract available.
Child* ; Humans

The polymerase chain reaction(PCR) was performed to detect clonal rearrangements of the immunoglobulin heavy chain gene(IgH) or T-cell receptor(TCR) genes in 57 lymphoproliferative diseases including 26 B-cell lymphomas and 23 peripheral T-cell lymphomas(PTCL). Using the IgH primers, monoclonality was detected in 19 out of 22(86.4%) cases of diffuse lymphomas and 2 out of 4(50%) follicular lymphomas, respectively, but not in the 23 PTCL cases. Using the V and J regions of the TCRgamma chain primers, monoclonality was detected in 14 out of the 23(60.9%) PTCL cases. TCR beta chain gene rearrangements were observed in 7 out of the 9 cases which did not show TCRgamma chain gene rearrangements. One non-T non-B acute lymphoblastic leukemia was found to have TCR beta chain gene rearrangements. In conclusion, analysis of Ig and TCR gene rearrangements by using the PCR technique can be used as a useful diagnostic adjunct to establish the clonality of various lymphoproliferative diseases.

In treatment of Vitamin D-resistant rickets, the authors supplemented supramalleolar stepcut osteotomy for complex deformity of tibia consisted of varus, anterior bowing and intemal torsional supramalleolar level which accompanied severe waddling gait. The results of 10 tibia operations from 5 patients were generally satisfactory in terms of appearance and function.
Congenital Abnormalities ; Gait ; Humans ; Osteotomy ; Rickets ; Rickets, Hypophosphatemic ; Tibia ; Vitamin D ; Vitamins

The perineum refers to the anatomical area immediately below the pelvis, and is comprised of diverse anatomic and somatic innervation. Although various interventions have been proposed for the management of intractable cancer-related perineal pain, their efficacy are limited because the target of nerve blocks has focused on somatic rather than sympathetic components. Recently, blockade of the ganglion impar has been introduced as an alternative means of managing intractable neoplastic perineal pain of sympathetic origin. We successfully treated a patient who had suffered from cancer-related intractable perineal pain with ganglion impar block.
Ganglion Cysts* ; Humans ; Nerve Block ; Pelvis ; Perineum ; Rectal Neoplasms

The clinical spectruni of epidermolysis bullosa acquisita(EBA) is much broader than originally thought. Although the full extent of the clinical presenation is still being defined, it is now known that EBA include the followings: a non-inflammatory mechanobullous condition equating wit,h classical EBA; an inflammatory vesiculosullous eruption mimicking bullous pernphigoid; and a mucosal-centered disease with sarring similar to cicatricial pemphigoid. Among the nine cases of EBA, aged between 34 to 70 year-old, seen in recent years, three patients had mechanobullous lesions with skin fragilities and scarrings; three patients had inflammatory bullous eruptions, and three other patient had combined features of mechanobullous/inflammatory bullous lesions. Mucous membrane lesions were recognized in sex cases, and the rnos! frequent site of involvement was the oral mucosae. According to observations of these patients episodes of inflammatory bullous eruptions appeared to be present in seven cases and have been considered as early sympoms of the disease. It has been noted, however, that in two cases lesions develop d as an non-inflammatory mechanobullous from thonset. Based on the ability of EBA to mimick bullous pemphigoid or cicatricial pernphogoid and the fact that such cases have perhaps been missed, we feel EBA is more common than past literature has suggested.
Aged ; Blister ; Cicatrix ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Mouth Mucosa ; Mucous Membrane ; Pemphigoid, Benign Mucous Membrane ; Pemphigoid, Bullous ; Skin

It has been increasingly clear that the defence against Mycobacterium leprae(M. leprae) appears mainly to depend on cell-mediated rather than humoral immune mechanism. Nevertheless, the M. leprae is not only capable of producing specific humoral antibody, but also stimulating the formation of a variety of autoantibodies, since mycobacteria are known to exert adjuvant effect. Although the exact role of the autoantibodies in the pathogenesis of leprosy is not known, it is remarkable that the prevalence of autoantibodies has been reported different by several investigators, suggesting the possibility of geographical or racial difference. This study was undertaken to investigate the prevalence of some autoantibodies in Korean patients with leprosy. Eighty patients with leprosy registered at the Department of Dermatology, Seoul National University Hospital entered this study from February, 1977 through October, 1978, The diagnosis was made by clinical, histological, bacteriological and immunological assessments and the patients were classified according to the Ridley-Jopling scale. All patients were under anti leprosy chemotherapy with DDS (Diaminodiphenylsulfone) for various periods at the time of study. Venereal Disease Research Laboratory (VDRL) test was performed in 80 patients and the sera displaying reactive VDRL were subjected to re-examination by Treponema Pallidum Hemagglutinin Assay (TPHA). Rbeumatoid factor was sought by means of latex fixation test in 66 patients. Antinuclear antibody (ANA) was detected by means of latex agglutination reaction in 61 patients using polysterene latex complexed with calf thymus deoxyribonucleoprotein. Cryoprotein was detected as described elsewhere. Four of the 80 patients(5%) showed reactive VDRL, while rheumatoid factor and antinuclear antibody were not detectable in all cases. Cryoprotein was detected in 15 patients (27. 3 %). Compared with other reports on the prevalence of autoantibodies in Caucacian and African patients, we found a much lower frequency. This result may be expained partly by the racial or geographic difference in the pattern of leprosy as suggeste4 by Turk.
Agglutination ; Antibodies, Antinuclear ; Autoantibodies* ; Dermatology ; Diagnosis ; Drug Therapy ; Hemagglutinins ; Humans ; Latex ; Latex Fixation Tests ; Leprosy* ; Mycobacterium ; Prevalence* ; Research Personnel ; Rheumatoid Factor ; Seoul ; Sexually Transmitted Diseases ; Thymus Gland ; Treponema pallidum

Apocrine carcinoma is a rare type of mammary cancer, which shows partial or total apocrine differentiation in either ductal or lobular carcinoma. The malignant transformation of apocrine epithelium of the breast was first described by Krompecher in 1916. It is well known that their relationship to true apocrine glands of the skin is only a morphological similarity, and this histological difference does not affect the prognosis. The authors experienced two cases of apocrine carcinoma of the breast which involved infiltrating ductal carcinoma of a 64-year old woman and intraductal carcinoma of a 69-year old woman respectively. Electron microscopic examination and brief review of literature was done.
Female ; Humans

Nodular-cystic fat necrosis is an unusual, distinctive entity characterized by histologically en-capsulated fat necrosis in the subcutis. We report two cases of nodular-cystic fat necrosis. The lesions were subcutaneous nodules. Histopathologically, the nodules showed strong periodic acid-Schiff -positive necrotic adipose tissue with lipomembranous changes and calcification.
Adipose Tissue ; Fat Necrosis*

No abstract available.
Child* ; Humans ; Proteinuria*

Tuberous sclerosis is a systemic disorder of hamartoma format on includes facial angiofibromas, subungal fibromas, hypomelanic maciles Shagreens patches. The earliest skin manifestation of tuberous sileiule, but it may appear later. We present a case of fibrous plaqu a recognized skin signs. Forehead plaque can be the earliest skin near in many organs. Skin involvement less forehead fibrous plaques, and vsis is usually hypornelanotic inacipearing before any of these more festation of tuberous sclerosis. Its presence may lead to early diagnosis and appropriate genetic couise hng hut may be a poor prognostic sign.
Angiofibroma ; Early Diagnosis ; Fibroma ; Forehead* ; Hamartoma ; Skin ; Skin Manifestations ; Tuberous Sclerosis*