1/34. Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha3.BACKGROUND: The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. patients with inflammatory EBA have IgG autoantibodies against type VII collagen. patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5. observation: We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha3. A final diagnosis of EBA was based on the sublamina densa level of blister formation. CONCLUSION: This case illustrates the clinical and immunological overlap between EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/34. Delayed and immediate hypersensitivity reactions associated with the use of amalgam.hypersensitivity to the constituents of dental amalgam is uncommon. When it occurs it typically manifests itself as a lichenoid reaction involving a delayed, type IV, cell-mediated hypersensitivity response. Rarely, a more acute and generalised response can occur involving both the oral mucosa and skin. We describe two cases that illustrate the presentation and management of these two types of reaction.- - - - - - - - - - ranking = 9.4497795061061keywords = mucosa (Clic here for more details about this article) |
3/34. Facial and perioral molluscum contagiosum as a manifestation of hiv infection.molluscum contagiosum (MC) is a self-limiting viral disease of the skin and the mucous membranes. Facial and perioral MC is seen with increasing frequency in human immunodeficiency (hiv) infection, particularly in hiv infected homosexual men. The purpose of this study was to describe clinical observations of facial and perioral MC in hiv infected patients. One hundred and eighty hiv-positive individuals (160 males and 20 females) were examined over a period of five years. Fifty-eight were homosexual men and 19 were bisexual men. Fifty-one of 180 patients at the time of the first examination had CD 4 count < 200 cells/mm3 and another 63 presented loss of CD 4 cells in this level, during this period. Three hiv infected patients (two homosexual and one bisexual) were affected with facial and perioral MC. At the time of MC diagnosis the CD 4 count was less than 200 cells/mm3 for all three patients. One patient died nine months after MC diagnosis and the other two are still alive. It is remarkable that in this study no clinical lesions were observed on other sites of the skin.- - - - - - - - - - ranking = 157.5419975336keywords = mucous membrane, membrane (Clic here for more details about this article) |
4/34. Late onset hyalinosis cutis et mucosae.Two cases of non-familial, late onset (50 and 62-years-old) hyalinosis cutis et mucosae were studied and compared with classical early onset cases. Late onset cases showed essentially the same histological and ultrastructural features described for early onset cases. The late onset variety should be distinguished from other diseases; they resemble systemic amyloidosis and sometimes the adult form of colloid milium.- - - - - - - - - - ranking = 47.248897530531keywords = mucosa (Clic here for more details about this article) |
5/34. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale.Eosinophilic angiocentric fibrosis (EAF) is an unusual fibrotic condition affecting the mucosa of the upper respiratory tract, of which there have been eight reported cases. The condition is thought to be associated with the rare skin disorder granuloma faciale, which is histologically identical, and was present in two cases of EAF. We report the third case where EAF and granuloma faciale occurred together, to highlight this type of intranasal fibrosis as a distinct pathological entity.- - - - - - - - - - ranking = 47.248897530531keywords = mucosa (Clic here for more details about this article) |
6/34. Hereditary complement (C9) deficiency associated with dermatomyositis.A 28-year-old Japanese woman with hereditary complement (C9) deficiency and dermatomyositis is reported. She had a 3-year history of facial erythema and a 1-month history of progressive muscle weakness. Clinical and laboratory findings were suggestive of dermatomyositis; muscle biopsy confirmed an inflammatory myopathy. An unexpected finding, however, was the low titre of serum haemolytic complement (CH50). Treatment with prednisolone resulted in marked clinical improvement but did not affect the CH50 titre. Further investigation revealed a selective and total absence of the ninth complement component (C9), with direct dna sequence analysis revealing a non-sense mutation at Arg95 of the C9 gene. This case demonstrates that the muscle lesions of dermatomyositis can occur in the presence of a complement defect that would prevent the formation of the C5b-9 membrane attack complex.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
7/34. Orofacial manifestations of histoplasmosis in hiv-positive patients: a case report.Amongst the main opportunistic diseases that affect the hiv-positive patient, histoplasmosis is found. This systemic mycosis caused by the fungus histoplasma capsulatum has the capacity to disseminate from the lung to the skin and oral mucosa. Oral lesions of histoplasmosis can be found with ulcerated or nodular aspect, being always very painful and infiltrating the mucosa. When they are present in the mouth, they strongly indicate the presence of some kind of immunosuppression. This study shows the disease's evolution in an hiv-positive patient, who presented several ulcerated lesions in the oral cavity and facial skin. The symptomatology and clinical aspects of the lesions were not specific for the disease, and due to this, the diagnosis was obtained by cytological smear and oral biopsy. The results of the exams defined the disseminated picture of the infection. The treatment plan involved the use of amphotericin b for the lesions' remission, and, following this, itraconazole was administered in the maintenance phase.- - - - - - - - - - ranking = 18.899559012212keywords = mucosa (Clic here for more details about this article) |
8/34. Autoimmune vesicles on the face and neck. A variant of Brunsting-Perry type localized bullous pemphigoid?We report a case of blistering disease presenting a unique distribution of vesiculobullous lesions on the face and neck which is similar to Brunsting-Perry type of localized bullous pemphigoid (BP). Histopathology of a lesional skin biopsy demonstrated a subepidermal blister. Direct immunofluorescence demonstrated a strong linear deposition of IgG and IgA to the basement membrane zone, and a faint staining for C3. However, circulating antibodies were not detected by indirect immunofluorescence and immunoblotting. And the patient did not develop atrophic scars and was a relatively young woman. This case might be a variant of Brunsting-Perry type of localized BP or localized epidermolysis bullosa acquisita, presenting the clinical heterogeneity of subepidermal blistering diseases.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
9/34. Cowden disease. Report of two additional cases.Two patients with Cowden disease, a syndrome consisting of cutaneous papules and nodules on the face, oral mucous membranes and dorsal aspects of the forearms and hands in association with tumours of the thyroid gland, breasts, gastro-intestinal tract, and female reproductive tract, are reported. This disease is important to the physician because recognition of these cutaneous lesions may allow early diagnosis of internal neoplasia.- - - - - - - - - - ranking = 157.5419975336keywords = mucous membrane, membrane (Clic here for more details about this article) |
10/34. Linear oro-facial lichen sclerosus.Lichen sclerosus is a depigmenting mucocutaneous disorder that most frequently affects the female genitalia. Lichen sclerosus affecting the oral mucosa is extremely rare. Oral lesions are asymptomatic but cosmetically unacceptable. We report here a case of lichen sclerosus presenting with a linear lesion over the nose that extended to involve the philtrum and the upper lip with intraoral extension up to the gingiva. Treatment with a short course of oral and intralesional corticosteroids resulted in partial resolution of the lesions.- - - - - - - - - - ranking = 9.4497795061061keywords = mucosa (Clic here for more details about this article) |
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