1/31. 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 = membrane (Clic here for more details about this article) |
2/31. A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180.Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
3/31. Mucosal involvement in a patient with lymphomatoid papulosis.We report a 38-year-old woman with lymphomatoid papulosis (LyP) who had nodular and papulovesicular lesions develop on the genital area and oral commissure. Mucosal involvement in LyP is a rare event, and its clinical relevance is still unknown. Aggressive therapies are not recommended in patients with LyP with either cutaneous or mucosal involvement.- - - - - - - - - - ranking = 4.300285392851keywords = mucosa (Clic here for more details about this article) |
4/31. Papular acantholytic dyskeratosis of the vulva.We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.- - - - - - - - - - ranking = 150.74733803705keywords = mucous membrane, membrane (Clic here for more details about this article) |
5/31. Malakoplakia involving the abdominal wall, urinary bladder, vagina, and vulva: case report and discussion of malakoplakia-associated bacteria.A 29-year-old woman presented with a 3-month history of multiple purulent discharging nodules involving her lower abdomen, vulva, and left thigh. Physical examination also disclosed vaginal nodules and a left pelvic mass. cystoscopy revealed multiple mucosal nodules and a perforation of the left vesical wall that appeared to communicate with the pelvic mass. Biopsies of the vesical and vulvar nodules revealed malakoplakia. Surgery and antibiotic therapy resulted in regression of all the lesions.- - - - - - - - - - ranking = 4.300285392851keywords = mucosa (Clic here for more details about this article) |
6/31. Amniotic membrane used for vulvar adhesion treatment.We report here a case of vulvar adhesion that was difficult to cure by estrogen only. We used amniotic membrane to be a barrier and after the operation, the symptoms of vulvar adhesion were resolved.- - - - - - - - - - ranking = 5keywords = membrane (Clic here for more details about this article) |
7/31. The Laugier-Hunziker syndrome.Laugier and Hunziker described a syndrome consisting of asymptomatic benign areas of hyperpigmentation affecting the lips, buccal mucosa and, in 50%, the fingernails. We report a 67-year-old woman with the clinical features of Laugier-Hunziker syndrome in association with vulval pigmentation. histology, immunohistochemistry and electron microscopy from the various areas of pigmentation on the body confirmed the benign nature of the pigmentation. We review potential causes of oral and genital pigmentation, and suggest an expansion of the original syndrome described by Laugier and Hunziker to include more widespread areas of benign hyperpigmentation, which may associated.- - - - - - - - - - ranking = 4.300285392851keywords = mucosa (Clic here for more details about this article) |
8/31. Amniotic membrane grafting to treat refractory labial adhesions postpartum. A case report.BACKGROUND: Labial adhesions are common in prepubertal children, but their occurrence postpartum is extremely rare. CASE: A woman developed labial adhesions after a vaginal delivery. Topical estrogen cream was ineffective. Simple excision was performed, but the adhesions reformed two weeks later. Finally, amniotic membranes were used to cover the labia minora after they were separated by excision. The wound healed without adhesion formation. CONCLUSION: Excision plus amniotic membrane grafting is effective for recurrent labial adhesions.- - - - - - - - - - ranking = 6keywords = membrane (Clic here for more details about this article) |
9/31. Vulvar lichen planus associated with ulcerative colitis. A case report.BACKGROUND: lichen planus (LP) is a common inflammatory dermatosis the diagnosis of which can be very complex when it affects the female genital area exclusively. The condition should be managed by both dermatologists and gynecologists because the association between vulvar LP and other immune-mediated disorders occurs frequently. CASE: Vulvar LP was diagnosed in 37-year-old woman whose past history was significant for ulcerative colitis (UC) since the age of 21. The oral mucosa and skin were completely unaffected, but whitish papules and plaques were seen diffusely on the whole vulvar region, which had become sclerotic and edematous. Microscopic examination of lesional tissue confirmed the diagnosis, showing classic histologic features in spite of the mixed and hypertrophic clinical variant. Oral therapy with cyclosporin at an initial dose of 250 mg/d led to complete remission within 3 months. CONCLUSION: This case is reported on for the rarity of isolated vulvar involvement by LP and for the excellent response to treatment.- - - - - - - - - - ranking = 4.300285392851keywords = mucosa (Clic here for more details about this article) |
10/31. Erosive lichen planus of the vulva and vagina.BACKGROUND: Erosive lichen planus causes erosion of the vulva and vagina and characteristic oral lesions. dyspareunia is usual, and vaginal stenosis may occur. This report highlights the clinical features and the response to medical therapy. CASES: We report the case histories of three women who presented to the Vulvovaginal Disorders Clinic of the University of iowa with long histories of dyspareunia and advanced vaginal scarring. In each case, the clinical diagnosis of erosive lichen planus was obvious but had not been made previously. All three women have responded well to topical treatment with tacrolimus 0.1% ointment. CONCLUSION: Erosive lichen planus should be suspected in a case of vaginal erosion or narrowing. Surgical management is inappropriate when the mucosa is eroded. Inspection of the mouth may confirm the diagnosis.- - - - - - - - - - ranking = 4.300285392851keywords = mucosa (Clic here for more details about this article) |
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