Epidermolysis Bullosa Pruriginosa

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منابع مشابه

Epidermolysis bullosa pruriginosa--report of three cases.

Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, is characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions, appearing at birth or later. Scarring and prurigo are most prominent on the shins. Treatment is unsatisfactory. We report three such cases: two of them f...

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Epidermolysis bullosa pruriginosa masquerading as psychogenic pruritus.

BACKGROUND Epidermolysis bullosa pruriginosa is a rare clinical subtype of dystrophic epidermolysis bullosa characterized by intense pruritus, secondary scratching-induced lesions, and pronounced scarring. OBSERVATIONS We describe a patient with epidermolysis bullosa pruriginosa who was misdiagnosed as having psychogenic pruritus for several years. Except for nail (toenail) dystrophy, no feat...

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Epidermolysis bullosa pruriginosa: a case with prominent histopathologic inflammation.

IMPORTANCE Epidermolysis bullosa (EB) pruriginosa is a rare variant of dystrophic EB. It may manifest late in life and is characterized by intense pruritus, resulting in a phenotype resembling acquired inflammatory dermatoses. Dermatopathology textbooks include hereditary forms of EB among the "cell-poor" list of subepidermal blistering disorders. OBSERVATIONS We report a case of dominant dys...

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Dystrophic epidermolysis bullosa pruriginosa in Italy: clinical and molecular characterization.

Dystrophic epidermolysis bullosa (DEB) pruriginosa (DEB-Pr) is a rare variant of DEB due to COL7A1 dominant and recessive mutations, which is characterized by severe itching and lichenoid or nodular prurigo-like lesions, mainly involving the extremities. Less than 30 patients have been described showing variable disease expression, and frequently, delayed age of onset. We report the clinical an...

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Successful treatment of epidermolysis bullosa pruriginosa with topical tacrolimus.

A 53-year-old white woman with epidermolysis bullosa (EB) pruriginosa presented to the dermatology clinic seeking therapy for localized severe and intractable pruritus. At the age of 4 years the patient developed an extremely pruritic bullous eruption characterized by fragile blisters, erosions, and excoriations affecting sites subject to friction and pressure, particularly the ankles and knees...

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ژورنال

عنوان ژورنال: JAMA Dermatology

سال: 2013

ISSN: 2168-6068

DOI: 10.1001/jamadermatol.2013.155