1/8. Childhood vulval pemphigoid: a clinical and immunopathological study of five patients.We describe five girls with vulval pemphigoid: two had bullous pemphigoid confined to the vulva and three had cicatricial pemphigoid. They demonstrate a spectrum of severity from localized disease to extensive vulval scarring necessitating long-term immunosuppressive therapy and surgical correction. The age at onset of their disease ranged between 6 and 13 years. All presented with vulval discomfort and erosions. Three had oral lesions, two perianal and one eye and cutaneous involvement. Two girls with only vulval lesions and one with vulval and oral lesions responded well to topical steroids. In two, systemic treatment with prednisolone and dapsone or azathioprine was required. The diagnosis was made on the basis of histology and immunofluorescence (IF). All had positive direct IF with IgG and C3. Indirect IF demonstrated circulating IgG binding to the basement membrane zone in four, with dermal or epidermal binding on salt-split skin substrate. immunoblotting revealed antibodies to the BP230 and BP180 antigens. Immunoelectron microscopy in the child with dermal binding IgG and BP180 and BP230 on immunoblotting showed labelling at the lamina densa-lamina lucida interface adjacent to hemidesmosomes.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/8. Langerhans cell histiocytosis of the vulva: an ultrastructural study.langerhans cells histiocytosis (LCH) is a proliferative disorder of langerhans cells. The lesions are normally characterized by infiltration of eosinophils, neutrophils, lymphocytes, plasma cells, and langerhans cells. The specific cells of LCH contain Birbeck granules, express the phenotype of langerhans cells but with markers fixed at an early stage of activation, and are functionally defective in antigen-presenting ability. The disease most often affects children; when it occurs in older patients, anal and groin involvement is quite common and vulvar lesions can be found in older females. The authors report a case of a 64-year-old woman with LCH of the vulva and diabetes insipidus. An immunohistochemical and ultrastructural study of the vulvar lesions showed an infiltrate in which antigenically and morphologically mature langerhans cells, monocytoid cells, and cells with an intermediate phenotype between monocytes and langerhans cells were concurrently observed. Although the clinical and histological aspects of LCH are well established, the pathogenetic mechanism of lesions is not yet known. The finding of an infiltrate composed by langerhans cells and many putative precursors of these cells suggests the hypothesis of an in situ differentiation of langerhans cells from immature monocytoid precursors.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/8. Localized childhood vulval pemphigoid treated with tacrolimus ointment.BACKGROUND: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. observation: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. CONCLUSION: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/8. Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid.BACKGROUND--Childhood localized vulvar pemphigoid has been recently reported in four girls. A fifth child with this proposed rare variant of bullous pemphigoid is described. Moreover, findings in the various immunopathologic studies we performed establish this entity as a true morphologic variant of bullous pemphigoid. OBSERVATIONS--In situ deposits of IgG in this patient's epidermal basement membrane zone localized to the epidermal side of 1 mol/L of saline-split skin. Moreover, the patient had circulating IgG autoantibodies that bound the epidermal side of 1 mol/L of saline-split skin in indirect immunofluorescence microscopy and immunoprecipitated the 230-kd bullous pemphigoid antigen from biosynthetically radiolabeled human keratinocyte extracts. These laboratory findings are identical to those documented in patients with the generalized "classic" form of bullous pemphigoid. CONCLUSIONS--This study demonstrates that a child with clinical, histopathologic, and immunopathologic features of localized vulvar pemphigoid had circulating autoantibodies that identify a specific keratinocyte antigen, the bullous pemphigoid antigen, which may serve as a molecular marker for this disease.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
5/8. A case of idiopathic vulvar calcinosis: the first in japan.Idiopathic calcinosis of the scrotum is a rare condition of unknown etiology. As counterparts of this male disorder, only two female cases have been reported. We report a third case, the first of its kind, in japan. Histochemical stains revealed acid mucopolysaccharide (acid-MPS) within the calcinosis and many infiltrated mast cells near it; these might histogenese the calcium deposition. Immunohistochemically, polyclonal carcinoembryonic antigen (CEA) and keratin stains revealed no positive cells near the calcium depositions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/8. behcet syndrome: with immunologic evaluation.A case of behcet syndrome with immunologic evaluation, including screening of a vulvar ulcer for IgG, IgM, IgA, and fibrinogen by direct fluorescent microscopy is presented. Attempts were made to demonstrate cellular and humoral immune responses to mucosal antigens by lymphoblast transformation in the presence of cadaver esophageal mucosal extracts and indirect immunofluorescence using autologous serum and mucosal tissue. Serial measurements of percentages of total T, active T, and B lymphocyte populations, and lymphocyte response to phytohemagglutinin (PHA) stimulation during the course of behcet syndrome are also presented. Clinical evaluation, histology of a Behcet vulvar ulcer, and a 2-year followup with good response to chlorambucil are reviewed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/8. An adult case of histiocytosis X with a vulvar ulcer and multiple bone lesions.A 62-year-old female with histiocytosis X presented with a vulvar ulcer. Multiple osteolytic lesions were later detected. Histological examination of the ulcerated skin showed diffuse proliferation of histiocytic cells with folded nuclei and pale eosinophilic cytoplasm. immunohistochemistry revealed S100 protein and vimentin as well as CD1a, CD4, and hla-dr antigens in the proliferating cells. Electron microscopy demonstrated Birbeck granules in the cytoplasm of the cells. The patient was successfully treated by complete surgical excision of the ulcer followed by radiotherapy for recurrent vulvar erythema.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/8. Bartholin's abscess complicating food poisoning with salmonella panama: a case report.A patient is presented who developed an acute Bartholin's abscess four weeks after an attack of salmonella panama enteritis. Aspirate from the abscess also grew salmonella panama, indistinguishable from the gut isolate in serotype and antigenic structure (1 9, 12: 1, v: 1,5). Some aspects of the microbiology of Bartholin's abscess and its clinical management are discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |