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Open Access T-lymphocytes are Directly Involved in the Clinical Expression of Migratory Circinate Erythema in Epidermolysis Bullosa Simplex Patients

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Epidermolysis bullosa simplex with migratory circinate erythema (EBS-MCE) is a rare EBS subtype characterised by migratory blistering lesions that resolve with brownish pigmentation. It is caused by a recurrent readthrough mutation, c.1649delG, in the tail of keratin 5. Here, we report a child with EBS-MCE and investigated the immunologic mechanisms underlying the migratory lesions in this patient. A skin biopsy from the patient from an active border of an erythematous lesion was used for the immunohistochemical characterisation of the inflammatory infiltrate and for TUNEL assay to detect apoptotic cells. We found abundant CD4+ and CD8+ T lymphocytes infiltrating the papillary dermis and lining the dermal–epidermal junction. A number of these cells expressed the activation marker CD69. CD83+ dendritic cells were present both in the epidermis and papillary dermis. Finally, TUNEL staining showed apoptosis of basal and suprabasal keratinocytes. These findings suggest a critical role of the cellular immunity in determining the EBS-MCE phenotype.

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Keywords: GENODERMATOSIS; INTERMEDIATE FILAMENTS; MOTTLED PIGMENTATION; NONHELICAL TAIL DOMAIN

Document Type: Research Article

Publication date: May 1, 2014

More about this publication?
  • Acta Dermato-Venereologica is a bi-monthly international peer-reviewed journal for clinical and experimental research in the field of dermatology and venereology.

    It was founded in 1920 by Johan Almqvist and edited during 1936-1969 by Sven Hellerström. Since 1970, the journal has been published for the Society for the Publication of Acta Dermato-Venereologica on a non-profit basis.

    Acta Dermato-Venereologica publishes high-quality manuscripts in English dealing with new observations on basic dermatological and venereological research, as well as clinical investigations. Each volume also features a number of review articles in special areas, as well as short Letters to the Editor to stimulate debate and to disseminate important clinical observations. Acta Dermato-Venereologica has rapid publication times and is amply illustrated with a large number of colour photographs to enhance understanding.

    Acta Dermato-Venereologica covers inter alia:

    • ~ Atopic dermatitis and contact allergy
    • ~ Facial dermatoses and adnexal disorders
    • ~ New treatments
    • ~ Psoriasis and genodermatoses
    • ~ Psychodermatology
    • ~ Sexually transmitted diseases
    • ~ Skin biology and inflammation
    • ~ Skin cancer and pigmentation
    • ~ Skin immunology and lymphoma
    • ~ Urticaria and itch

    Extensive papers, proceedings from congresses and symposia are printed as supplements to the journal. Announcements concerning pertinent dermatological meetings and books received are also published.
    Readership: Doctors and scientists interested in dermatology, skin biology and venereology.

    Acta Dermato-Venereologica has an impact factor of 3.007.

    Issues between 1998 up to 2005 can be found at http://www.medicaljournals.se/acta/content

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