1/356. Unusual location of an ovary: ultrasonographic features and surgical correlation. We present a case of ectopic ovary in a 5-month-old baby presenting a firm nodule in the left labia majora. ultrasonography was performed and revealed an ectopic ovary. The ectopic ovary was surgically returned in adnexial location. We describe the typical findings of this entity. ( info) |
2/356. Primary breast carcinoma of the vulva: a case report and literature review. BACKGROUND: In 1872, Hartung was the first to describe the case of a fully formed mammary gland arising in the left labium majora of a 30-year-old woman. Since Hartung's initial report, 38 additional cases of ectopic vulvar breast tissue have been described. This case report describes the rare occurrence of primary mammary adenocarcinoma arising within the vulva. CASE: A 64-year-old G4P4 white female presented with a 4-year history of a 2 x 1 cm firm, indurated, raised lesion of the left lateral mons. A wide local excision with ipsilateral inguinofemoral lymphadenectomy was performed. Given histological findings characteristic of both invasive ductal carcinoma and invasive lobular carcinoma, in conjunction with the presence of estrogen and progesterone receptors within the tumor, a diagnosis of infiltrating adenocarcinoma arising within ectopic breast tissue was made. CONCLUSIONS: Thirty-nine reported cases of ectopic breast tissue arising within the vulva have been reported in the world literature. Though the diagnosis of primary breast carcinoma arising within the vulva is based primarily upon histologic pattern, estrogen and progesterone receptor positivity provide supporting evidence. Given the rarity of this condition, guidelines for therapy are unavailable; we therefore suggest looking to the current management of breast cancer in order to establish a sensible approach. ( info) |
3/356. Accidental vaccinia of the vulva. vaccinia of the vulva in a 32-year-old married woman is described. The vaccination was apparently due to a heteroinoculation from her husband during sexual contact. Clinically it presented as an indurated ulcer with a few isolated umbilicated vesicles and was associated with an acute biological false-positive serological reactions. ( info) |
| vulvodynia is a problem most family physicians can expect to encounter. It is a syndrome of unexplained vulvar pain, frequently accompanied by physical disabilities, limitation of daily activities, sexual dysfunction and psychologic distress. The patient's vulvar pain usually has an acute onset and, in most cases, becomes a chronic problem lasting months to years. The pain is often described as burning or stinging, or a feeling of rawness or irritation. vulvodynia may have multiple causes, with several subsets, including cyclic vulvovaginitis, vulvar vestibulitis syndrome, essential (dysesthetic) vulvodynia and vulvar dermatoses. Evaluation should include a thorough history and physical examination as well as cultures for bacteria and fungus, KOH microscopic examination and biopsy of any suspicious areas. Proper treatment mandates that the correct type of vulvodynia be identified. Depending on the specific diagnosis, treatment may include fluconazole, calcium citrate, tricyclic antidepressants, topical corticosteroids, physical therapy with biofeedback, surgery or laser therapy. Since vulvodynia is often a chronic condition, regular medical follow-up and referral to a support group are helpful for most patients. ( info) |
| We report the first case of vulvar porokeratosis that occurred in a 39-year-old woman with a 30-year history of vulvar pruritus and disfiguring vulvar lesions. Ultrasonic surgical aspiration resulted in resolution of her pruritus and excellent cosmesis. light microscopy revealed characteristic cornoid lamellae and electron microscopy confirmed the diagnosis of vulvar porokeratosis. ( info) |
6/356. Vulvar paraneoplastic amyloidosis with the appearance of a vulvar carcinoma. Nodular cutaneous amyloidosis of the vulva is a rare phenomenon. We describe a patient with localized nodular lesions on the vulva that mimicked kissing ulcers such as are seen with vulvar carcinoma. These lesions were a result of multiple myeloma with subsequent primary systemic amyloidosis. The patient died of cardiac and renal decompensation 2 months after diagnosis. ( info) |
7/356. 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. ( info) |
8/356. Bockenheimer's syndrome. A case of a very extensive form of the rare Bockenheimer's syndrome (genuine diffuse phlebectasia) in a 45-year-old female patient is presented. The subject presented phlebectasias of the upper right leg, the vulva and the right side of the abdomen. The diagnostic and therapeutic possibilities for cases of patients with Bockenheimer's syndrome are described. ( info) |
9/356. 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. ( info) |
10/356. 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. ( info) |