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      Epidermolysis bullosa pruriginosa: A report of two cases

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          Abstract

          Epidermolysis bullosa (EB) pruriginosa is a very rare pattern of dystrophic EB caused by type VII collagen gene mutation, with distinctive clinico-pathological features. It is characterized by nodular prurigo-like lichenified lesions, nail dystrophy, and variable presence of albopapuloid lesions. We report two such cases.

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          Most cited references 13

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          Epidermolysis bullosa pruriginosa: dystrophic epidermolysis bullosa with distinctive clinicopathological features.

          We report a study of eight unrelated adult patients with a highly distinctive phenotype of dystrophic epidermolysis bullosa. It is characterized clinically by pruritus, lichenified or nodular prurigo-like lesions, violaceous linear scarring, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and, in some cases, albopapuloid lesions on the trunk. The scarring is most evident on the limbs, particularly on the shins, with relative sparing elsewhere. Intact blisters are rarely seen. Physical signs were present at birth in three patients, but in the others skin manifestations were first noticed between 6 months and 10 years of age. Five cases are sporadic, but three of the eight patients have a history of familial involvement, with autosomal dominant inheritance in two cases and recessive transmission in the other case. Studies of the dermal-epidermal junction showed alterations in the number and ultrastructure of anchoring fibrils in lesional, perilesional and non-lesional skin, consistent with a diagnosis of dominant or localized recessive dystrophic epidermolysis bullosa. These patients represent an unusual, poorly recognized form or expression of dystrophic epidermolysis bullosa which has features in common with a variety of acquired inflammatory dermatoses.
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            Thalidomide in the management of epidermolysis bullosa pruriginosa.

            Epidermolysis bullosa (EB) pruriginosa is a distinctive clinical subtype of dystrophic EB. We report a patient with dominant dystrophic EB pruriginosa, who had an excellent response to systemic thalidomide treatment. The mechanism of action of thalidomide in the management of pruriginous disorders is not yet completely understood. Most recent studies point towards an immunomodulatory action of thalidomide that may suppress excessive production of tumour necrosis factor-alpha and may downregulate certain cell surface adhesion molecules involved in leucocyte migration.
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              Successful treatment of epidermolysis bullosa pruriginosa with topical tacrolimus.

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                Author and article information

                Journal
                Indian Dermatol Online J
                Indian Dermatol Online J
                IDOJ
                Indian Dermatology Online Journal
                Medknow Publications & Media Pvt Ltd (India )
                2229-5178
                2249-5673
                Jan-Mar 2014
                : 5
                : 1
                : 44-47
                Affiliations
                Department of Dermatology, Goa Medical College Bambolim, Goa, India
                [1 ]Consultant Dermatologist, Gadag, Karnataka, India
                [2 ]Dermatology, SDM College of Medical Sciences and Hospital, Sattur, Dharwad, India
                [3 ]Pathology, SDM College of Medical Sciences and Hospital, Sattur, Dharwad, India
                Author notes
                Address for correspondence: Dr. Varadraj V Pai, Department of Dermatology, Goa Medical College, Bambolim, Goa - 580 009, India. E-mail: docpai@ 123456rediffmail.com
                Article
                IDOJ-5-44
                10.4103/2229-5178.126030
                3937487
                Copyright: © Indian Dermatology Online Journal

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Dermatology

                bullosa, epidermolysis, pruriginosa

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