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

No abstract available.
Giant Cell Tumors* ; Giant Cells* ; Tendons*

Amyloidosis is a disease complex associated with deposition of inscluble fibrillar protein in vnrious tissues of the body. Since the term, amyloidosis was first introduced by Virchow in 1853, there have been many reports in English literature, but only a few cases of iriinary systemic amyloidosis have been reported in Korea. A 56-year-old male was seven for facial purpuras, macroglossia, myilgia and arthralgia for 5 years. Histologically, the skin biopsy specimen showed amorphous, faintly eosinophilic and fissured masses of amyloid in the upper dermis that demonstrated characteristic green brefringence on Congo red staining when viewed under polarized light. Electron microscopic exanintion showed that nonbranching and nonanastomosing straigh. fibrils are irregularly arranged arouned tlie collagen fibers. Therefore, he was diagncsed with primary systemic amyloidsis by the characteristic clinical, histopathologic and ultrastructural findings.
Amyloid ; Amyloidosis* ; Arthralgia ; Biopsy ; Collagen ; Congo Red ; Dermis ; Eosinophils ; Humans ; Korea ; Macroglossia ; Male ; Middle Aged ; Purpura ; Skin

There are many known methods of dermo-epidermal separation for the investigation of autoimmune blistering diseases. Investigators should select a proper method since many differences exist preservation of antigenicity. In order to determine the stabilization of antigenirity by different separation methods, we have separated dermo-epidermal junction by means of 1M s;ilt, 56C PBS, 20mM EDTA and dispase. Indirect immunofluarescence and immunoblotting were performed on each specimen with sera of patients with pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, bullous pemphigoid and epidermolysis bullosa acquisita. The results are as follows : 1. In indirect immunofluorescence study of pemphigus group, best, result were obtained when normal skin without dermo-epidermal separation was used. Dispase well preserved antigenicity of pemphigus after dermo-epidermal separation, but no differences were noted in antigenicity stabilization among separation mehods by immunoblotting. 2. In indirect immunofluorecence study for differentiation of bullous pemphigoid and epidermolysis bullosa acquisita, we recommend EDTA and dispase methods in addition to 1M salt induced skin separation that have been most popularly used. 3. Results of the immunoblotting of bullous pemphigoid showed that 1M salt, EDTA and heat preserved the antigenicity well but the antigenicity was lost by dispase. 4. Results of the immunoblotting of epidermolysis bullosa acquisita she wed that antigen did not exist in epidermal extract. 5. Antigen preservation according to the different methods of demo-epidermal separation was not identical between indirect immunofluorescence and immunoblotting.
Blister* ; Edetic Acid ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Indirect ; Hot Temperature ; Humans ; Immunoblotting ; Pemphigoid, Bullous ; Pemphigus ; Research Personnel ; Skin

BACKGROUND: Preservation of antigen determinants while retaining morphological detail is prerequisite for high quality immunohistochemistry. Conventional formalin fixation and paraffin embedding procedures are useful in preserving tissue architecture and cytologic detail. However, they destroy the antigenicity of many proteins is tissue samples. On the other hand, fresh frozen section preserve the antigenicity of most proteins, but vield poor morphological preservation. OBJECTIVE: The purpose of this study is to evaluate the AMeX method as to the ability to preserve both antigenicity and morphologic details of the skin basement membrane zone so that precise localization of antigens can be attained in immunohistochemistry. METHODS: Tissues were fixed in acetone at -20degrees C over night, then cleared in methyl benzoate and xylene, consecutively, and embedded in ordinary paraffin at 58-60degrees C. Sections made from this paraffinembedded tissue were stained with hematoxylin and eosin for a morphologic study and immunolabelled with antibodies against major basement membrane antigens to evaluate antigenic preservation. The staining intensity and preservation of the morphology by the AMeX method were compared with conventional formalin processed tissues and frozen tissues. RESULTS: Morphological preservation of the AMeX method-processed sections was good throughout the epidermis, basement membrane, and dermis, and as good as that of routinely formalin-fixed paraffin-embedded sections. Frozen sections usually revealed revealed various degrees of damage by ice crystal formation throughout the epidermis to the dermis. The AMeX method-processed sections showed better or same antigenic preservation comparing the frozen sections when the sections were immunolabelled with specific monoclonal antibodies. But, when the sections were immunolabelled with patient's sera, the AMex method showed less antigenic preservation than the frozed sections. The anti-type IV collagen monoclonal antibody exhibited immunoreactivity only conventional formalin-fixed paraffin-embedded skin sections, but the intensity of the staining was weaker than the AMeX processed sections and the frozen sections. CONCLUSION: The AMeX method can be utilized for the demonstration of skin basement membrane antigens and is superior to the fresh-frozen method in that the histologic figures are more distinct and antigencity can be preserved for a long time.
Acetone* ; Antibodies ; Antibodies, Monoclonal ; Basement Membrane* ; Benzoates* ; Collagen ; Dermis ; Eosine Yellowish-(YS) ; Epidermis ; Formaldehyde ; Frozen Sections ; Hand ; Hematoxylin ; Ice ; Immunohistochemistry ; Paraffin ; Paraffin Embedding ; Skin* ; Xylenes*

Pseudo-Kaposis sarcoma is a self-limited process associated with vascular malformations, without the neoplastic character of real Kaposis sarcoma. In most cases, it has been associated with an underlying congenital arterial venous fistula and chronic venous insufficiency, We present a case of Pseudo-Kaposis's sarcoma developing after plagment of arteriovenous fistula for hemodialysis in a patient wiith chronic renal failure and review cases reported in articles.
Arteriovenous Fistula ; Fistula* ; Humans ; Kidney Failure, Chronic ; Renal Dialysis* ; Sarcoma* ; Sarcoma, Kaposi ; Vascular Malformations ; Venous Insufficiency

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital pigmented nevi and a benign or maigrant pigment cell tumor of the leptomeninges. The s ndrome is thought to represent an erroi in the morphogenesis of the embryonal neuroectoderm. Even in the absence of melanorra, symptomatic neurocut.aneous melanosis has extremely poor prognosis. We report a case of neiirocutaneous melanosis in 38-year-old male. He was noted at birth to have a giant pigmented nevus on the bathing trunk area. He presneted with severe headache. Brain CT sca n was performed and 4 x 4cm sized well demarcated contrast enhanced mass was seeii in the right frontal lobe. Histopathologically, the tumor was composed of malignant melanoma cells. Multiple skin biopsies werperformed on the giant pigmented nevus and small nevi. All of the specimens revealed patterns typical of cornpound nevi. Dermatologists following patients with large or multiple congenial pigmented nevi should be aware of this condition to aid in prompt diagnosis.
Adult ; Baths ; Biopsy ; Brain ; Diagnosis ; Frontal Lobe ; Headache ; Humans ; Male ; Melanoma ; Melanosis* ; Morphogenesis ; Neural Plate ; Nevus ; Nevus, Pigmented ; Parturition ; Prognosis ; Skin

In a previous communication, we described a relatively simpli. method for assaying quantities of sweat using the skin surface hydrometer. In order to evaluate the pharmacologic response of weat glands in psoriasis, vitiligo and loalized scleroderma, we measured the basal skin conductance and the quantity of sweat induced by intradermal injections of pilocarpine nitrate in the lesional and per ilesional skin. The results were as follows : 1. In psoriatic plaques, based skin surface hydration and the sweat response to pilocarpine was markedly reduced (p<0.05), but there were no significant differences in normal perilesional skin. 2. In generalized vitiligo, basal skin surface hydration and the sweat gland response was normal. In the case of the segmental type, there was a slightly decreased sweat response in the lesion but, not significant statistically. 3. In the morphea plaque, basal skin surface hydration and the sw.at response to pilocarpine was markedly reduced but was normal in perilesional skin. This study presents evidence of the functional changes in the development of sweat secretion and surface hydration in the lesions of psoriasis, morphec and segmental type of vitiligo. The demonstration of ahnormalities in the pharmacolopic response to pilocarpine induction led us to suggest the possibility that some degree of sweat gland involvement is induced by the pathogenetic mechanisms of these diseases.
Injections, Intradermal ; Pilocarpine ; Psoriasis* ; Scleroderma, Localized ; Skin ; Sweat Glands* ; Sweat* ; Vitiligo*

No abstract available.
Emergencies* ; Emergency Service, Hospital* ; Humans ; Statistics as Topic*

No abstract available.
Glomerulonephritis*