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1/40. Linear IgA disease histopathologically and clinically masquerading as lichen planus.

    In each of 2 cases reported, the patient presented with features of erosive lichen planus or lichenoid drug eruptions and an incisional biopsy taken from the patient was diagnosed histologically as lichen planus. Subsequent recurrences or exacerbations were associated with vesiculobullous lesions. Simultaneous or subsequent direct immunofluorescence studies--from the same tissue sample in one case and from a similar site in the other case--demonstrated classic features of linear IgA disease. Both patients were originally treated for lichen planus with systemic and/or topical corticosteroids with limited success. One patient was treated with sulfapyridine with minimal improvement. Both patients were subsequently treated with dapsone and demonstrated significant clinical improvement. We propose that linear IgA disease may be more common than reported in the oral cavity, inasmuch as many cases of recalcitrant lichen planus, erosive lichen planus, and lichenoid drug eruptions, especially those with a vesiculobullous component, may in reality represent linear IgA disease. We recommend that direct immunofluorescence be done in any case in which bullous lichen planus is suspected.
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ranking = 1
keywords = vesiculobullous, bullous
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2/40. Acute (toxic) epidermal necrolysis. Report of a case.

    A case of acute (toxic) epidermal necrolysis is reported in the dental literature for the first time. This severe, life-threatening mucocutaneous syndrome resembles severely scalded skin. Sloughing vesiculobullous oral lesions are a frequent component. staphylococcus aureus and drug reactions may cause separate entities presenting clinically as acute epidermal necrolysis. This case of drug-induced epidermal necrolysis ina child was successfully treated with methicillin and hydrocortisone.
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ranking = 0.49930475507783
keywords = vesiculobullous, bullous
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3/40. Treatment of recalcitrant cicatricial pemphigoid with the tumor necrosis factor alpha antagonist etanercept.

    The treatment of cicatricial pemphigoid is generally regarded as difficult and usually relies on individual clinical experience. Corticosteroids, as drugs of first choice, often have to be combined with steroid-sparing agents to prevent hazardous, long-term side effects. We describe a 72-year-old woman with long-standing cicatricial pemphigoid recalcitrant to established treatment regimens who responded rapidly and lastingly to therapy with the tumor necrosis factor alpha antagonist etanercept. To our knowledge, this is the first report of its use in the treatment of a bullous autoimmune disease.
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ranking = 0.0013904898443318
keywords = bullous
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4/40. Benign mucous membrane pemphigoid--a case report.

    Benign mucous membrane pemphigoid (BMMP) is a relatively rare, chronic vesiculobullous disease. It frequently affects postmenopausal women, although cases have been reported in younger individuals. Benign mucous membrane pemphigoid has a predilection to affect multiple mucosal surfaces including the gingivae, hard and soft palate, alveolar ridge, nose, pharynx, gastrointestinal tract, genitalia and the conjunctiva. It is seen clinically as bullae or erosions on the mucosae or gingivae. The bullae rupture after 24-48 hours and the erosions heal within 7-14 days, sometimes with scar formation. Scarring frequently occurs with ocular mucosa involvement and may contribute to blindness. In order to make a diagnosis, the clinical features must be correlated with microscopic and immunopathological findings. Benign mucous membrane pemphigoid is treated with high doses of corticosteroids and immunosuppressive agents. This paper is a presentation of a case report in a 36-year-old woman.
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ranking = 0.49930475507783
keywords = vesiculobullous, bullous
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5/40. Angina bullosa haemorrhagica: presentation of eight new cases and a review of the literature.

    Angina bullosa haemorrhagica (ABH) describes the acute and sometimes painful onset of oral blood-filled vesicles and bullae not attributable to blood dyscrasia, vesiculo-bullous disorders, systemic diseases or other known causes. The haemorrhagic bullae spontaneously burst after a short time resulting in ragged, often painless, superficial erosions that heal spontaneously within 1 week without scarring. Although the pathogenesis is still unclear, ABH seems to be a multifactorial phenomenon: dental or functional trauma seems to be the major provoking factor. The lesions of ABH can be easily confused with other mucosal diseases. It is important that the presentation of this benign disorder is distinguished from other more serious disorders with similar presenting features. The aim of this paper is to report the clinical features of eight cases of ABH, in an attempt to distinguish ABH from other blistering diseases of oral mucosa and to describe their management.
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ranking = 0.0013904898443318
keywords = bullous
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6/40. Paraneoplastic pemphigus: a report of three cases including one long-term survivor.

    BACKGROUND: Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. OBJECTIVE: We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. methods: We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. RESULTS: All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19 , CD5 B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. CONCLUSION: We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.
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ranking = 0.0013904898443318
keywords = bullous
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7/40. pemphigus vulgaris and complications of systemic corticosteroid therapy: a case report.

    pemphigus refers to a group of diseases characterized by painful lesions caused by intraepidermal acantholytic structures in the skin and mucous membrane. The exact nature of the disease remains unknown. pemphigus is a rare chronic mucocutaneous disease characterized by intra-epithelial bulla formation, due to autoantibodies directed against proteins of the desmosome-tonofilament complex between keratinocytes. The bullous lesions are painful, slow to heal and with a tendency to become invasive. Any part of the oral cavity may be affected, with the soft palate, buccal mucosa and lips being the most common sites. The high doses and prolonged administration of corticosteroids often required to control the disease result in several side effects, many of which are serious or life-threatening. In the present case, steroid treatment was begun at 180 mg/day and subsequently increased to 250 mg/day and 350 mg/day. Oral lesions were treated locally with 0.2% chlorhexidine gluconate in addition to systemic corticosteroids containing an immunosuppressive. Oral lesions were observed to recur without healing completely. Respiratory problems occured, necessitating direct lung radiographs and computerised tomography. pulmonary embolism was diagnosed and the patient was transferred to the vascular surgery department. Unfortunately the patient died due to pulmonary embolism on the seventh day. pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease that often primarily involves the oral cavity. Therefore, early diagnosis of oral symptoms is crucial for the successful treatment of PV. Although there is no consensus regarding the initial steroid dosage needed to induce remission, it is suggested that high doses of corticosteriods may cause fatal complications.
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ranking = 0.0013904898443318
keywords = bullous
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8/40. Oral and cutaneous lichen planus pemphigoides.

    lichen planus pemphigoides is a rare bullous disorder characterized by tense bullae on lichen planus lesions and on clinically uninvolved skin. A diagnosis of lichen planus pemphigoides is made on the basis of clinical, histologic, and immunopathologic evaluation. We describe a patient who had lichen planus pemphigoides of the skin and oral mucosa and briefly review the literature on this uncommon entity.
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ranking = 0.0013904898443318
keywords = bullous
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9/40. oral manifestations of papular-purpuric 'gloves and socks' syndrome due to parvovirus B19 infection: the first case presented in greece and review of the literature.

    Papular-purpuric 'gloves and socks' syndrome (PPGSS) is a novel, rare, self-limited dermatosis initially described in 1990. It is characterized by painful, pruritic edema and erythema, rapidly evolving to papular-purpuric lesions on the distal extremities, in a gloves-and-socks distribution, accompanied by fever and oral lesions such as petechiae, vesiculopustules and small erosions. parvovirus B19 has been implicated in most cases as the etiological factor. Herein we present the first case of PPGSS in a 42-year-old Greek man with von Willebrand disease. On admission the patient was febrile, and presented acral edema and erythema rapidly followed by purpuric lesions on the same sites, and palatal petechiae. Complete remission of the exanthem occurred 7 days after hospitalization. Clinical and laboratory evaluation including serologic tests and PCR, confirmed the presence of parvovirus B19. review of the existing literature on this novel syndrome and its association with parvovirus B19 is also presented.
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ranking = 0.00057475364342404
keywords = dermatosis
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10/40. Oral psoriasis in an 11-year-old child: a case report.

    psoriasis is a chronic inflammatory dermatosis that very rarely involves the oral mucosa. There is no consistent pattern of clinical presentation in the oral cavity. Furthermore, manifestations of intraoral psoriasis in paediatric patients are extremely rare and only one case appeared to have been reported in the literature to date. In this paper we report a case of intraoral psoriasis in an 11-year-old child who had been previously diagnosed as having dermal psoriasis.
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ranking = 0.00057475364342404
keywords = dermatosis
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