BACKGROUND: Desmosomes are adhesive intercellular junctions that form an important component of the junction complexes of epithelial cells. They provide intercellular links between the intermediate filament cytoskeletons of adjacent cells and are thus involved in maintaining the structural integrity of tissues. OBJECTIVE: Calcium and retinoids are major regulators of epidermal differentiation and their role on keratin proteins are well known. However, their effects on desmosome moleucles are unknown. To address this question we initiated a study of the effects of these epidermal differentiation regulators on desmosomal components, i.e., desmoplakin, desmoglein, and pemphigus antigens. METHODS: We used monoclonal antibodies against desmoplakin(DP) and desmoglein(DG), and sera from patients with pemphigus vulgaris(PV), pemphigus foliaceus(PF) and paraneoplastic pemphigus (PNP) to study the effects of calcium and retinoic acids, which are major regulators of epidermal differentation, on desmosomal protein formation in human cultured deratinocytes. We performed immunofluorescence, immunoblotting and immunoprecipitation study using human keratinocytes cultured in high calcium media with or without retinoic acid and in low calcium media with or without retinoic acid. RESULTS: 1. In low calcium (0.15mM) media, PV antigen and DG were produced in a small amount and it appeared that these desmosomal proteins were located in cytosol. Whereas in high calcium (1.8mM) media, production of these desmosomal proteins was increased not they were assembled at the desmosomal structures located in cell-cell contact margins. 2. PF antigen, which was identical to the DG, were not produced or expressed in cultured keratinocytes even when cultured in high calcium media. 3. PNP antigen and DP were produced in cultured keratinocytes grown in both high low calcium media but their production was increased in high calcium media and only in high calcium media they were assembled at the desmosomal structures. 4. Retinoic acids induced loosening of cell-cell contacts of cultured keratinocytes and decreased the production of desmosomal proteins. CONCLUSION: Our results suggests calcium is a major regulator of the production and assembly of desmosomal proteins including pemphigus antigens, but PF sera and monoclonal antibodies against DG show different antigen binding characteristics. It appears that retinoic acids inhibit production of desmosomal proteins.
Adhesives ; Antibodies, Monoclonal ; Calcium* ; Cytoskeleton ; Cytosol ; Desmogleins ; Desmoplakins ; Desmosomes* ; Epithelial Cells ; Fluorescent Antibody Technique ; Humans ; Immunoblotting ; Immunoprecipitation ; Intercellular Junctions ; Intermediate Filaments ; Keratinocytes ; Pemphigus ; Retinoids ; Tretinoin*

A 67-year-old man noted a 4×6cm sized, erythematous, erosive, oozing patch on the penile shaft, which resisted conservative therapy for three years. Histopathologic examination revealed typical findings of extramammary Paget's disease. Interestingly, nests of Paget's cells in some hair follicular structures were shown in the middermis. The lesion was excised by microscopically controlled excision, using the fresh-tissue technique. There were histologically involved areas extending to quite a distance beyond the clinically normal appearing skin. Mohs micrographic surgery might offer the unique advantage of effective surgical treatment with microscopically controlled tumor free borders in the extramammary Paget's disease.
Aged ; Hair ; Humans ; Mohs Surgery ; Paget Disease, Extramammary* ; Skin

l6-year-old male and 48-year-old female with systemic lupus erythematosus (SLE) and renal disease presented with vesiculobullous eruptions during severe attack of the disease. They showed hypocomplementernia, and high ANA titers. Histopathologic findings revealed subepidermal blister and leukocytoclastic vasculitis, Direct immunofluorescence demonstratedlinear deposits of IgG, IgA and lgM at the dermoepidermal junction. The vesiculobullous eruption of SLE may be an important marker reflecting disease activity and prognosis.
Blister ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Lupus Erythematosus, Systemic* ; Male ; Middle Aged ; Prognosis ; Vasculitis

BACKGROUND: Pemphigus are chronic autoimmune blistering disorcers characterized by acantholysis. In addition to pemphigus vulgaris(PV), the major clinical variarts are pemphigus foliaceus(PF), paraneoplastic pemphigus(PNP) and drug-induced pemphigus(DP). Detection of pemphigus antigen is important for differential diagnosis as well as research work. Most investigators have identified pemphigus antigens by means of immunoprecipitation using metabolically radiolabeled cultured keratinocytes. However, immunorepitation is generally more expensive, hazardous and time-consuming than immunoblotting. Therefore, establishment of the immunoblotting as a standard technique for the detection of the pemphig us antigens is desirable. OBJECTIVE: To characterized pemphigus antigens by an immunobloting analysis of human epidermal extract and by indirect itnmunofluroscence study using human of cultured keratinocytes as a substraie. METHOD: We performed imrnunoblotting analysis af sera from patieiits with PV, PF, PNP and DP with human epidermal extract as a source of antigen. Indirect immunof uorescence study was also performed using human keratinocytes cultured in high or low calcium media for detection of pemphigus antigens. RESULTS: In an immunoblotting analysis, all(9/9) PV sera showed secific reactivities with a 130-KD protein and all(5/5) FF sera showed reactivities with a 150-KK protein, which is most likely desmoglein 1. Furthermore, one of nine PV serum also reacted with a 150-KD protein, which seems to be the identical antigen detected in PF. All PNP(3/3) sera showed reactivities with two protein bands, 210KD and 190KD. In our indirect imrnunofluorescence study using culltured human keratinocytes as a substrate, when keratinocytes were grown in low calcium media, no pimphigus antigens could be detected. However, when grown in high calciurn media, pemphigus vulga ris and paraneoplastic pernphigus antigens were present t the cell-cell contact areas with a puncta;e pattern, whereas pemphigus foliaceus antigen was not, presint in keratinocytes even when cultured in high calcium media. CONCLUSION: Our results suggests (1) immunoblotting analysis is a reliable technique for defining pemphigus antigen and could be a valuable tool for the differentiation of PV, PF and PNP and(2) PF antigen rnay not be expresseden cultured keratinocytes.
Acantholysis ; Blister ; Calcium ; Desmoglein 1 ; Diagnosis, Differential ; Fluorescent Antibody Technique, Indirect* ; Humans ; Immunoblotting ; Immunoprecipitation ; Keratinocytes* ; Pemphigus* ; Research Personnel

We report herein a case of eosinophilic pustular follicultis in a 20-year-old man. The patient showed typical clinical picture with specific laboratory and histopathological findings of eosinophilic pustular folliculitis. The patient responded well to systemic administration of corticosteroid and dapsone.
Dapsone ; Eosinophils* ; Folliculitis* ; Humans ; Young Adult

Maffucci's syndrome is a congenital, non-hereditary mesodermal d splasia manifested by multiple enchondromas and hemangiomas. Several case reports and reviews indicate that there is a tendency toward malignant transformation of the enchondroma. The incidence of malignancy may be as high as 30%. We report a case of 10-year-old female with multiple hemangiomas on the face, palms, and soles and skeletal chondromatoses of the multiple bones.
Child ; Chondroma ; Chondromatosis ; Enchondromatosis ; Female ; Hemangioma ; Humans ; Incidence ; Mesoderm

Aneurysm of the internal carotid artery is a rare disease and is known to be associated with congenital arterial anomalies such as neurofibromatosis type I (NF-I). NF-I is an autosomal dominant neurocutaneous disorder characterized by a variety of manifestations that involve the central and peripheral nervous systems, skin, vascular system, and skeleton. In particular, the involvement of vascular abnormalities in NF-I is well known. Any vessel may be affected by this condition, although the renal artery is most frequently involved. The vascular abnormality can be occlusive or an aneurysmal degenerative change. Therefore, symptomatic presentations might assume an indolent pathophysiologic course such as hypertension, or manifest as a catastrophic event such as arterial rupture that could result in sudden death. We report a rare autopsy case of an aneurysmal rupture of the internal carotid artery in a woman with suspected NF-I, who collapsed in her home.
Aneurysm ; Autopsy ; Carotid Artery, Internal ; Death, Sudden ; Female ; Glycosaminoglycans ; Humans ; Hypertension ; Neurocutaneous Syndromes ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nervous System ; Rare Diseases ; Renal Artery ; Rupture ; Skeleton ; Skin

17,966 new outpatients who visited the Department of Dermatology of Wonju Christian Hospital from 1985 to 1989 were analyzed statistically. The study results are summarized as follws : 1. Among the 17,966 outpatients, the total number of male patients were 8358 (46.5%) and female patients were 9608 (53.5%). 2. In age distribution, the most frequent age groups were lst decade (24.0), 2nd decade (16.5), and 3 rd decade (25.5) amounting to 66% of the total outpatients. 3. The 15 most common dermatoses were dermatophytosis, atopic dermatitis, urticaria, acne, seborrheic dermatitis, allergic contact dermatitis, other eczema, hypopigmentary disorder, bacterial infection, psoriasis, insect bites, and herpes zoster. 4. Those dermatoses which showed increasing tendency were other eczema, hypopigmentary disorder, herpes zoster, and those of decreasing tendency were scabies, bacterial infection. psoriasis, insect bites. 5. Seasonal distribution. showed those diseases occurring most frequently during the summer season were dermatophytosis, insect bites. urticaria, acne, eczema, contact dermatitis. hypopigmentary disorder, bacterial infection. and herpes zoster. The incidence of atopic deratitis. and psoriasis were incfreased during the winter season. 6. In sexual distribution sebourrheic dermatitis. and scabies were more frequent in the males. whereas with urticaria. acne, contact dermatitis, and other eczema the females were more commonly affected. 7. In age distribution, atopic dermatitis, bacterial infection, and insect bites were the most frequent in the Ist decade. Dermatophytosis. urticaria, acne, seborrheic dermatitis, contact dermatitis, other eczema, hypopigmentary disorder, drug eruption, and psoriasis were most frequent in the 3 rd decade, with scabies in the 2nd decade, and herpes zoster in the 6th decade. 8. The distributions of dermatoses as disease groups, were eczema (23.8%), dermatophytosis (13.9%), urticaria-drug eruption (12.2%), skin appendage disorder (11.2%), and viral infection (8.1%), which constituted 69.2% of the total.
Acne Vulgaris ; Age Distribution ; Bacterial Infections ; Dermatitis ; Dermatitis, Allergic Contact ; Dermatitis, Atopic ; Dermatitis, Contact ; Dermatitis, Seborrheic ; Dermatology ; Drug Eruptions ; Eczema ; Epidemiology ; Female ; Gangwon-do* ; Herpes Zoster ; Humans ; Incidence ; Insect Bites and Stings ; Male ; Outpatients ; Psoriasis ; Scabies ; Seasons ; Skin ; Skin Diseases* ; Statistics as Topic* ; Tinea ; Urticaria

No abstract available.
Purpura, Thrombocytopenic* ; Rifampin*

A case of peripheral neuroblastoma of a 33-year-old male, which was located on the left buttock, is presented. Histologcally, this tumor demonstrated smaliound cell infiltrations which were arranged in a characteristic rosette pattern and the tumor cells were positively stained with neuron specific enolase. We review the clinical, histopathological ultrastructural and immunohisto chemical characteristics of this rare tumor, as well as the differential diagnosis with other small round cell tumors.
Adult ; Buttocks ; Diagnosis, Differential ; Humans ; Male ; Neuroblastoma* ; Phosphopyruvate Hydratase