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epidermolysis bullosa acquisita/erythema

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Epidermolysis bullosa acquisita in identical twins.

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Identical twin sisters developed generalized erythema and bullae on skin and mucous membranes at 18 and 19 years of age. Atrophic scars and milia were formed later. Indirect immunofluorescence (IF) study of the separated skin by incubation in 1.0M NaCl showed antibasement membrane zone (BMZ)
We report a patient with a spectrum of clinical features simulating toxic epidermal necrolysis, bullous erythema multiforme and later, dermatitis herpetiformis (DH). The histological features were suggestive of DH, bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA). Direct

Human orf complicated by epidermolysis bullosa acquisita.

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Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after orf infection, including erythema multiforme. A few cases of autoimmune bullous dermatosis complicating orf disease have been reported to date. They are usually
Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially

Bullous pemphigoid with prominent milium formation.

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Milia are very common superficial keratinous cysts, clinically seen as pearly white dome-shaped lesions with a diameter of 1-2 mm. Bullous pemphigoid (BP) is an autoimmune bullous disease characterized clinically by tense bullae on the extremities and trunk. The major target autoantigens of BP are

Non-infectious diseases of the oral soft tissue: a new approach.

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A significant proportion of the non-infectious diseases of oral mucosa are either auto-immune in nature or have lesions which are the result of immunologically-mediated events. These include pemphigus, benign mucous membrane pemphigoid, linear IgA bullous dermatosis, dermatitis herpetiformis,

Hodgkin's disease with specific bullous lesions.

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A variety of bullous eruptions have been reported in patients with Hodgkin's disease, among them erythema multiforme, herpesvirus infections, bullous impetigo, prurigo-like papules with vesicles, drug eruptions, bullous pemphigoid, dermatitis herpetiformis, and acquired epidermolysis bullosa. We now

When to request immunofluorescence: practical hints.

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This review highlights important considerations in obtaining good skin biopsy specimens to optimize results of direct immunofluorescence (IF) studies and also summarizes the various patterns of cutaneous IF deposition and their associated diagnoses. IF findings of immunobullous diseases, lupus

Psoriasis bullosa acquisita.

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We report a 51-year-old man with a 20-year history of chronic plaque psoriasis who developed an autoimmune subepidermal blistering eruption that had clinical features of bullous pemphigoid, erythema multiforme and epidermolysis bullosa acquisita. Investigations revealed a 1 : 400 titre circulating
Caterpillar bodies are eosinophilic, elongated, segmented bodies located within the roofs of blisters and are considered to represent a specific histopathologic feature of porphyric bullous eruptions, including porphyria cutanea tarda and erythropoietic protoporphyria. The possibility that similar

Dermatological Manifestations in Pediatric Inflammatory Bowel Disease

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Background and Objectives: Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist.

Oral mucosal diseases: the inflammatory dermatoses.

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The oral inflammatory dermatoses is a term used to describe a number of predominantly immune-mediated disorders: lichen planus (LP), erythema multiforme (EM), the vesiculobullous diseases pemphigoid (MMP), pemphigus (PV) and epidermolysis bullosa acquisita (EBA). These conditions are characterized

Cutaneous manifestations of gastrointestinal disease: part II.

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The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II

Mucous membrane pemphigoid and oral blistering diseases.

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The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history-taking, clinical examination, an understanding of pathogenesis and appropriate
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