1/9. 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 = epidermis (Clic here for more details about this article) |
2/9. epidermal cyst of the clitoris: a rare cause of clitorimegaly.Epidermal cysts are slowly growing, intradermal or subcutaneous tumors with a wall composed of true epidermis. They occur most commonly on the face, scalp, neck, and trunk. We report the unusual case of a 16-year-old girl with an epidermal cyst of the clitoris. The tumor was removed by local excision.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
3/9. Benign persistent papular acantholytic and dyskeratotic eruption of the vulva: a case report.We report a case of 44-year-old woman with persistent pruritic papules on the left and right labium majus of the vulva. Histopathologic examination of the vulvar biopsy specimen revealed a suprabasal separation of the epidermis with acantholysis and dyskeratosis.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
4/9. Acantholytic dermatosis of the vulvocrural area.Acantholytic dermatosis of the vulvocrural area is a rare skin disorder characterized by solitary or multiple skin-colored to white, smooth papules or plaques. Histopathological features of both Hailey-Hailey disease and Darler's disease are present. There is acantholysis, which may involve the full thickness of the epidermis, and dyskeratosis with corps ronds and grains. There may be marked hyperkeratosis and focal parakeratosis. We report a case of this rare disease and discuss its differential diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
5/9. Pseudoepitheliomatous hyperplasia in lichen sclerosus of the vulva.Small tentacles or separated nests of squamous cells in the dermis are not uncommonly seen in long-standing vulvar lichen sclerosus (LS) associated with epidermal thickening. We recently encountered a case where separated nests of well-differentiated squamous cells in the dermis were difficult to distinguish from squamous cell carcinoma (SCC). Further biopsies showed similar nests originating from every hair follicle. We postulated a diagnosis of multifocal pseudoepitheliomatous hyperplasia (PEH) to explain this phenomenon. Because we could find no reference to PEH in the setting of LS, we reviewed the biopsies of 92 women with extragenital and vulvar LS with and without carcinoma to determine its frequency and histological appearance. The study population, which excluded the index case, comprised 10 women with extra-anogenital LS, 58 with vulvar LS without carcinoma, and 24 with vulvar LS with carcinoma. The presence of PEH, epidermal thickness, predominant dermal collagen change, degree of inflammation, and presence of fibrin and red blood cells were recorded. The presence or absence of lichen simplex chronicus (LSC), squamous cell hyperplasia (SCH), and differentiated vulvar intraepithelial neoplasia (VIN) were recorded. PEH was identified only in vulvar LS, where it was seen in 7/58 (12.1%) women without carcinoma, 1/24 (8.3%) with carcinoma, and 0/10 (0%) with extra-anogenital LS. Two forms of PEH were seen: predominantly epidermal 7/8 (87.5%) and predominantly follicular 1/8 (12.5%). PEH was associated with increased epidermal thickness, less dermal edema, more dermal inflammation, fresh fibrin, and red blood cell extravasation. In all cases, there was associated LSC, but there was no SCH or differentiated VIN. In conclusion, PEH may explain many of the cases of dermal tentacles and separated squamous nests in vulvar LS with LSC. The association with fresh fibrin and red blood cells suggests that PEH might be a reaction to tissue damage. PEH is distinguished from SCC by its lack of atypia, confinement to the abnormal collagen, and limited growth. The pathologist must be careful about making a diagnosis of PEH in LS with epidermal thickening, looking carefully for basal atypia and other features of differentiated VIN in the overlying epidermis or dermal proliferation. We do not know whether PEH occurs in differentiated VIN and, if it does, how it could be distinguished from SCC.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
6/9. Papular acantholytic dyskeratosis of the vulva.We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years follow-up, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
7/9. A case of acantholytic dermatosis of the vulva with features of pemphigus vegetans.We report an unusual case of vulvar acantholytic dermatosis with features of pemphigus vegetans in a 22-year-old Indian girl who presented with a "warty" lesion in her left labium majus. Following excision of this lesion, she presented with 2 localized recurrent lesions on the left and right labia majora about 2 1/2 years later which were also excised. All 3 biopsies showed histological features typical of pemphigus which included extensive suprabasal acantholysis with bullae formation, prominent villus-like processes at the base of the bullae, focal hyperkeratosis and papillomatosis, and the occasional mixed neutrophil and eosinophilic intraepidermal abscess. IgG and C3 immunofluorescence was positive in the intercellular spaces of the epidermis. These lesions, which probably represent a form of pemphigus vegetans, have not been previously reported as a cause of localized vulvar acantholytic dermatosis.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
8/9. Ulcerating rheumatoid nodule of the vulva.A case of an ulcerating rheumatoid nodule of the vulva in a 76 year old woman with rheumatoid arthritis complicated by Felty's syndrome is reported. The patient presented with a mass in the vulval region. On clinical examination, she had an ulcerated mass associated with inguinal lymphadenopathy. These findings resulted in a clinical diagnosis of invasive carcinoma of the vulva and an excision biopsy was carried out. On microscopic examination, the lesion showed the characteristic features of a rheumatoid nodule with ulceration of overlying epidermis. Adjacent vessels showed inflammation and fibrinoid necrosis of their walls suggestive of a vasculitis. awareness of the possibility of ulceration in rheumatoid nodules may facilitate diagnosis and avert unduly aggressive treatment.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
9/9. A case of extramammary Paget's disease mimicking pemphigus vulgaris on histologic examination.We present a case of extramammary Paget's disease (EMP) on the vulva of a 77-year-old female that on histologic examination resembled pemphigus vulgaris. The diagnosis of EMP was established with the help of a periodic acid-Schiff stain and immunophenotyping. Ultrastructural studies revealed no desmosomes between tumor cells and keratinocytes and only rare desmosomes between adjacent tumor cells. A high number of tumor cells with scant desmosomes may be the ultrastructural correlate with cellular discohesion in the lower epidermis and with the histologic resemblance to pemphigus vulgaris.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |