epidermolysis bullosa/edema

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Upper airway and external genital involvement in epidermolysis bullosa dystrophica.

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Radiographic findings in two patients with uncommon manifestations of epidermolysis bullosa dystrophica are described. A girl with recurrent urinary tract infections had scarring of the external genitalia producing chronic vaginal and uterine reflux and retention of urine. A second patient with

[Herpetic-like worsening of an epidermolysis bullosa simplex during pregnancy].

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BACKGROUND Episodes of hereditary epidermolysis bullosa simplex are usually provoked by repeated cutaneous traumas or exposure to heat. We report a case of hereditary epidermolysis bullosa simplex worsened during pregnancy. METHODS A 21 year-old woman suffering from hereditary epidermolysis bullosa

Passive transfer of autoantibodies from a patient with mutilating epidermolysis bullosa acquisita induces specific alterations in the skin of neonatal mice.

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BACKGROUND Epidermolysis bullosa acquisita is a subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen in anchoring fibrils. These autoantibodies are believed to play an important role in the pathogenesis of sub-lamina densa blister formation in this

Bullous congenital ichthyosiform erythroderma masquerading as dystrophic epidermolysis bullosa.

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A black baby, born at full term of a normal healthy mother, showed multiple erosions and ulcers. No history of skin diseases was known in the family. A skin biopsy taken shortly after birth from an arm lesion showed subepidermal edema and cavity formation. Electron microscopy showed clumping of

Epidermolysis bullosa simplex in the Collie dog.

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Epidermolysis bullosa simplex was diagnosed in 4 Collie dogs. Three of the dogs were less than 6 months old when lesions were first noticed. Lesions were characterized by alopecia, erythema, edema, erythematous plaques, erosions, ulcers, crusts, atrophy, pigmentary changes, and (rarely) blisters

Ocular changes in epidermolysis bullosa letalis.

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Ocular changes in a rare case of epidermolysis bullosa letalis included edema of the trabecular meshwork, ciliary body, lens, optic nerve, and formation of edema cysts in the iris. The retinal was completely detached and focally necrotic. Foci of necrosis and detachment of the retinal pigment

Diffuse membranoproliferative glomerulonephritis with focal sclerosis and renal amyloidosis in an adult male with autosomal dominant dystrophic epidermolysis bullosa: a case report.

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Previous reports of glomerular disease in adult patients with autosomal dominant dystrophic epidermolysis bullosa (EB) are limited and include post-infectious glomerulonephritis, IgA nephropathy, amyloidosis, and leukocytoclastic vasculitis. To our knowledge, membranoproliferative glomerulonephritis

Epidermolysis bullosa acquisita and inflammatory bowel disease.

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Immunologic studies on lesional and nonlesional "normal" -appearing skin of a 32-year-old man with epidermolysis bullosa acquisita (EBA) revealed the following: (1) Noninflammatory subepidermal bullae formation in the periodic acid-Schiff (PAS)-positive basement membrane; (2) linear deposits of

Collagen VII deficient mice show morphologic and histologic corneal changes that phenotypically mimic human dystrophic epidermolysis bullosa of the eye.

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BACKGROUND Absence of collagen VII causes blistering of the skin, eyes and many other tissues. This disease is termed dystrophic epidermolysis bullosa (DEB). Corneal fibrosis occurs in up to 41% and vision loss in up to 64% of patients. Standard treatments are supportive and there is no cure. The

Conditional depletion of mast cells has no impact on the severity of experimental epidermolysis bullosa acquisita.

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The role of mast cells (MCs) in autoimmunity is the matter of an intensive scientific debate. Based on observations in different MC-deficient mouse strains, MCs are considered as fundamental players in autoimmune diseases. However, most recent data suggest that the outcome of such diseases is

Pathology of an ulcerative dermatitis in Belgian Landrace sows.

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Chronic ulcers of the skin were observed in three Belgian Landrace sows. Lesions were located on ears, limbs, and in the mammary region and were resistant to treatment that included corticosteroid therapy. Major histologic changes were located at the dermo-epidermal junction. Ulcers were deep, and

Human bullous pemphigoid antigen 2 transgenic skin elicits specific IgG in wild-type mice.

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Bullous pemphigoid antigen 2 (BPAG2) is targeted by autoantibodies in patients with bullous pemphigoid (BP), and absent in patients with one type of epidermolysis bullosa (OMIM #226650). A keratin 14 promoter construct was used to produce transgenic (Tg) mice appropriately expressing human BPAG2

Bullous systemic lupus erythematosus.

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Blistering eruptions are rare cutaneous manifestations of lupus erythematosus (LE) that may be caused by different mechanisms. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic-, subacute-, and acute-cutaneous LE due to a severe vacuolar alteration of the

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