The complement system is known to be involved in the pathogenesis of the skin lesions in pernphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, and systemic lupus erythematosus. Authors examined the skin specimens of each disease cases, who did not show any evidence of complement deficiency, to determine the deposition of complement components(C4, C3, Chb-9) and their inhibitors(C4bp, Factor H, S-protein) by modified direct immunofluorescence. We also looked at the staining pattern and localization, for further insights of their pathobiologic contributions in each disease. The findings of deposits of complement components up to C9, as well as inhibitor proteins at the primary histopathologic sites, in the majority of those cases, may indicate that the complement system, to certain extent, involves the inflamrnatory reactions in these diseases. The co-localization of C5b-9 and S-protein could be regarded as the consequence of in situ formation of SC5b-9 complexs or as the result of non-lytic adsorbed complexes of fluid phase SC5b-9. The pathologic role of the complement seems to depend mostly on the complement-fixing biologic property and the amount of the tissue bound immune complexes, which are often heterogeneous to different diseases and among different patients.
Antigen-Antibody Complex ; Complement Factor H ; Complement Membrane Attack Complex ; Complement System Proteins* ; Dermatitis Herpetiformis ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Humans ; Lupus Erythematosus, Systemic ; Pemphigoid, Bullous ; Skin ; Skin Diseases*

Pemphgus vulgaris (PV), Bullus pemphigoid (BP), and Epidermolysis bullous acqusita (EBA) are autoimmune bullous dermatoses, characterized by circulating IgG autoantibodies. These antibodies react with antigens located at the intercellular substance (ICS) of epidermis, basement membrane zone (BMZ), and subepidermal anchoring fibril zone (AFZ), respectively. The subclass distribution of IgG autoantibodies, and the properties and degrees of complement fixing activities of these autoantibodies in each of the above diseases have not been well understood. Indirect immunofluorescence and in vitro complement stainings were performed for the titration of subclasses of IgG antibodies and for the immunofluorescence staining reactivities of complement components C3, C4, C5b-9, H, C4bp, and S. Each serum specimen from five cases of PV, five cases of BP. and three cases of EBA was tested. The findings of multistep technique with monoclonal and polyclonal antibodies are as follows : All four subclasses of IgG antibodies were identified at the antigenic sites in these group, however there were some differences in the antibodies titers. In PV and BP the dominant subclass of highest antibody titer was IgG1 and/or IgG4. In EBA only IgG4 was dominant in all three cases. The results of complement component stainings, in most of the cases of PV, showed positive for C3 and C4 but were negative for the other components or inhibitor proteins at the ICS of epidermis. In BP most of the cases revealed positive staining reactivities at the BMZ for C3, C4, C5b-9, H, and C4bp-9 with no staining reactivities for the inhibitor proteins No significant relevancy was found between the titers of complement fixing IgG subclasses and the numbers of positive complement staining reactivities for complement components. The results suggest that the complement system may contribute more strongly to the formation of bullous lesions in BP and EBA than in PV.
Antibodies ; Autoantibodies ; Basement Membrane ; Blister ; Complement Membrane Attack Complex ; Complement System Proteins ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Immunoglobulin G ; Pemphigoid, Bullous* ; Pemphigus* ; Skin Diseases, Vesiculobullous

The delayed blanch is one of the most discussed but undecided facets of atopic dermatitis as yet. At present, they favor the theory that delayed blanch is due to vasoconstriction, but it is not obvious whether this is due to "true" vasoconstriction or to the blood vessels being narrowed by the surrounding exudative edema, resulted from vasodilatation and increased capillary per meability. We compared cutaneous response of severe atopic dermatitis with that of age matched non-atopic individuals after intradermal injection of acetylcholine by means of naked eye and laser Doppler flowmeter examination, control group showed flare and wheals while at,opy group resulted in delayed blanch in addition to flare and whealing. The results of laser Doppler flowmeter examination revealed that, as compared to baseline, atopy group showed statistically significantly increase in spite of delayed blanch. We suggested that the delayed blanch may be not due ta va.soconstriction but due to the vasadilatation and aceumulation of edema fluid.
Acetylcholine ; Blood Vessels ; Capillaries ; Dermatitis, Atopic* ; Edema ; Flowmeters ; Injections, Intradermal ; Vasoconstriction ; Vasodilation

Malignant histiocytosis(MH) is a rapidly progressive and usally fatal disorder characterized clinically fever, generalized weakness, lymphadeno athy, hepatosplenomegaly accompanied by jaundice, and purpura. The frequency of skin manifestations has been repoterd about 15% of MH patients. Papules, plaques and niidules are commonly met. Histopathologically MH shows infiltrations of atypical histiocyte; with uarious maturity in many organs including skin. We present a case of a 32-year-old female with MH involving not only lymph node, liver, spleen and bone marrow but also skin with vaorious features ; erythematous papules, annular purpuric patches, subcutaneous nodules and hemomagic nodules. Immunohist,ochemically the majority of patients atypical histiocytes in lesional skin shows a-1 antitrysin positive, but lysozyme negative. It may suggest that these are poorly differentiated atypical histiocytes rather than well differentiated ones.
Adult ; Bone Marrow ; Female ; Fever ; Histiocytes ; Histiocytic Sarcoma* ; Humans ; Jaundice ; Liver ; Lymph Nodes ; Muramidase ; Purpura ; Skin Manifestations* ; Skin* ; Spleen

Saccharomyces is an ascospore-producing yeast that is commonly employed in the brewery and bakery industries. We report a case of opportunistic skin infection with Saccharomyces in a 62-year-old female whose defense was impaired by immunosuppression. Previously reported cases have been treated with amphotericin B or ketoconazole and our patient respbnded to fluconazole.
Amphotericin B ; Female ; Fluconazole ; Humans ; Immunosuppression ; Ketoconazole ; Middle Aged ; Saccharomyces* ; Skin* ; Yeasts

Lymphocytic infiltration of the skin(LIS), first described in 1953 by Jessner and Kanot is relatively uncommon disorder, characterized clinicaily by the presence of erythemat.ous peripherally enlarging papules or plaques which may show central clearing. Histologically, the lesions show patch, perivascular and periadnexal lymphocytic infiltrates. We have experienced a case of 30-year old male who had multiple erythematous nodules and plaques on the face and dorsum of the right wrist with 5 years history. He had first noted erythematous plaques on the cheek and these lesions were gradually increasing in size and spreading to the forehead and forearm. He had transiently improved by medication at the drug store, but recurred after discontinuation.
Adult ; Cheek ; Forearm ; Forehead ; Humans ; Male ; Skin* ; Wrist

The origin of Paget cells in extramammary Pagets disease is uncertain, however, some ultrastructural surveys revealed differentistion of Paget cells toward sweat gland apparatus. This is supported by recent immunohistochemical studies that Paget cells are irnmunohistochemically shown to react with antikeratin monoclonal antibodies specific to simple epithelia. In this siudy, seven cases of extramammary Pagets disease were immunohistochemically investigated with adenokeratin, carcinoembryonic antigen(CEA), involucrin by peroxidase-antiperoxidase(PAP) techniques. The results were as follow : l. Epidermis, hair follicies and sebaceous glands revealed negative immunoreacativity for adenokeratin in all cases. Sweat glands(6/7), Paget cells(6/7) and underlying adenocarcinoma revealed positive immunoreactivity for adenokeratin. 2. Epidermis, hair follicles and sebaceous glands(6/7) revealed negative immunoreactivity for CEA. Sweat glands, Paget cells and underlying adenocarcinoma revealed positive immunoreactivity for CEA in all cases. 3. Lower epidermis, sweat glands(6/7), Paget cells and underlying adenocarcinorna revealed negative irnmunoreactivity for involucrin. Upper epidermis, hair follicles and sebaceous glands(5/7) revealed positive immunoreactivity far involucrin. Based on these results, Paget cells of extramammary Pagets disease are considered to be derived from the sweat glands.
Adenocarcinoma ; Antibodies, Monoclonal ; Carcinoembryonic Antigen* ; Epidermis ; Hair ; Hair Follicle ; Paget Disease, Extramammary* ; Sebaceous Glands ; Sweat ; Sweat Glands

We report a case of angiocentric lymphoma in a 41-year-old female who complained chiefly of a genital ulcer and a bulging conjunctiva on the right eye. She had a history of a re-current oral ulcer over a 5 year period and a genital ulcer over 3 years. The above manifested clinical findings may suggest Behcet's disease. However, a biopsy from the upper vaginal wall and the inferior turbinate showed that atypical small and large lymphocytes with hyper-chromatic, elongated and convoluted nuclei had aggregated around the thickened blood vessels, which is consistent with angiocentric lymphoma. Immunophenotypically, the atypical cells were positive for pan T-cell markers not B-cells. Serologically, the patient showed a high titer of i?B virus viral capsid antigen(VCA) IgG.
Adult ; B-Lymphocytes ; Biopsy ; Blood Vessels ; Capsid ; Conjunctiva ; Female ; Humans ; Immunoglobulin G ; Lymphocytes ; Lymphoma* ; Oral Ulcer ; T-Lymphocytes ; Turbinates ; Ulcer

Langerhans sell bistiocytosis(LCH) is a reactive disease in which abnomal Langerhans cells accurnulate in various body sites and cause damage to affected organs. LCH usually occurs in childrea bur can also affect adults although rarely. LCH in a case of 65-year-old man initially involwed the lymph nodes (left supraclacicular and right inguinal area) and pelvis bone, and eight months later curancous involvement was noted. The skin lesions were waxy from the waxy papules showed that the dense infiltrate, ptedominantly of histiocytes, was present in the dermis, Immunohistochemistry for S-100 protein showed positive staining snd electron miscoroscopy disclosed Birbeck granules, which is characteristic findings of Langerhans cells.
Adult* ; Aged ; Dermis ; Histiocytes ; Histiocytosis, Langerhans-Cell* ; Humans ; Immunohistochemistry ; Langerhans Cells ; Lymph Nodes ; Pelvis ; S100 Proteins ; Skin

BACKGROUND: When cells or organisms are exposed to environmental stresses, they respond by synthesizing a characteristic group of proteins called heat shock proteins(HSP) or stress proteins. In a variety of HSP, the so-called HSP 70 family is the most prominent, conserved, and best characterized. The HSP 70 family is required for survival of cells during and after thermal stress. OBJECTIVE: The purpose of this study is to investigate if the cultured human melanocytes and rnelanotic malignant melanoma cell lines(SK 30) expressed HSP 70 family unstressed, after heat shock and ultraviolet exposure. METHODS: Protein was isolated from melanocytes and SK 30. Western blotting was done for identification of the HSP 70 family. RESULTS: HSP 70 family expression could be detected in the unstressed cultured human melanocytes and SK 30(malignant melanoma cell lines). HSP 70 family expression inereased in the melanocytes and SK 30 after heat shock. Irradiation of the melanocytes with UVA resulted in a decrease in expression of HSP 70 family after 32, 48 J/cm compared with 4, l6 J/cm. Irradiation of the melanocytes with UVA + B resulted in a dose-dependent increase in expression of HSP 70 family but a decrease in expression of HSP 70 family after 80mJ/cm. Irradiation of SK 30 with UVA resulted in a dose-dependent decrease in expression of the HSP 70 family. CONCLUSION: HSP 70 family expression was detected even unstressed. This high base line HSP 70 family expression may suggest that melanocytes have ability to protect from environmental stresses like keratinocytes.
Blotting, Western ; Heat-Shock Proteins* ; Hot Temperature* ; HSP70 Heat-Shock Proteins* ; Humans ; Keratinocytes ; Melanocytes* ; Melanoma ; Shock