Subcorneal Pustular Dermatosis

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Jeanjacques Bonerandi - One of the best experts on this subject based on the ideXlab platform.

T Hashimoto - One of the best experts on this subject based on the ideXlab platform.

  • Rapid response of IgA pemphigus of Subcorneal Pustular Dermatosis type to treatment with isotretinoin.
    Journal of the American Academy of Dermatology, 2020
    Co-Authors: C Gruss, D Zillikens, T Hashimoto, M Amagai, M Kroiss, T Vogt, M Landthaler, W Stolz
    Abstract:

    Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA Dermatosis type and Subcorneal Pustular Dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with Subcorneal Pustular Dermatosis type of IgA pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA pemphigus. Isotretinoin was an effective drug in the treatment of Subcorneal Pustular Dermatosis type of IgA pemphigus in this patient.

  • superficial neutrophilic dermatoses from Subcorneal Pustular Dermatosis sneddon wilkinson disease to intercellular iga dermatoses
    2018
    Co-Authors: Laurence Feldmeyer, T Hashimoto, Luca Borradori
    Abstract:

    Since the initial description of the Subcorneal Pustular Dermatosis by Sneddon and Wilkinson in 1956 [1], our understanding of the pathophysiology of diseases associated with the accumulation of neutrophils in the epidermis and intraepidermal blistering has significantly evolved. Sneddon-Wilkinson disease is now regarded as condition belonging to the spectrum of neutrophilic dermatoses characterized by neutrophilic infiltration of the skin and potential extracutaneous involvement, such as Sweet syndrome and pyoderma gangrenosum [2–4]. This group of inflammatory conditions, which show considerable clinical and histological overlap, is frequently associated with systemic diseases. Recently, it has been suggested to use the term of superficial neutrophilic dermatoses for all neutrophilic diseases associated with intraepidermal neutrophil accumulation [5]. Intriguingly, there is a group of patients who develop a superficial neutrophilic disease very similar to Sneddon-Wilkinson disease, showing either Subcorneal or intraepidermal pustule formation. However, in contrast to Sneddon-Wilkinson disease, these patients show characteristically IgA deposits on the cell surface of epidermal keratinocyte in a pemphigus-like pattern or more rarely linear deposits in the Subcorneal zone.

  • Subcorneal Pustular Dermatosis type iga pemphigus with autoantibodies to desmocollins 1 2 and 3
    Archives of Dermatology, 2009
    Co-Authors: Imke Duker, D Zillikens, T Hashimoto, Jorg Schaller, Christian Rose, Johannes Kunze
    Abstract:

    Background IgA pemphigus is a rare neutrophilic acantholytic autoimmune disease that is characterized by IgA deposits on keratinocyte cell surfaces. Clinically and histologically, IgA pemphigus is divided into 2 major subtypes: Subcorneal Pustular Dermatosis and intraepidermal neutrophilic IgA Dermatosis. We report the first case of Subcorneal Pustular Dermatosis–type IgA pemphigus that showed reactivity to all 3 isoforms of the desmocollin family by indirect immunofluorescence microscopy of COS7 cells transfected with desmocollin 1, 2, or 3. Observations We describe a 94-year-old woman with IgA pemphigus with a unique immunopathologic pattern. Direct immunofluorescence microscopy revealed IgA deposits throughout the entire epidermis, with stronger staining in the upper epidermis. The autoantibodies from this patient did not show IgA or IgG reactivity with desmogleins via immunoblotting or enzyme-linked immunosorbent assay. By indirect immunofluorescence by the use of COS7 cells transfected with desmocollin 1, 2, or 3, IgA autoantibodies in a serum sample from our patient clearly reacted with all of them. Conclusions The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially pemphigus and its subforms, are more complex than previously believed. Because pemphigus seems to be a heterogeneous disorder, further studies are needed to evaluate the complexity of the disease.

  • rapid response of iga pemphigus of Subcorneal Pustular Dermatosis type to treatment with isotretinoin
    Journal of The American Academy of Dermatology, 2000
    Co-Authors: C Gruss, D Zillikens, T Hashimoto, M Amagai, M Kroiss, T Vogt, M Landthaler, W Stolz
    Abstract:

    Abstract Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA Dermatosis type and Subcorneal Pustular Dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with Subcorneal Pustular Dermatosis type of IgA pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA pemphigus. Isotretinoin was an effective drug in the treatment of Subcorneal Pustular Dermatosis type of IgA pemphigus in this patient. (J Am Acad Dermatol 2000;43:923-6.)

  • Subcorneal Pustular Dermatosis type of iga pemphigus demonstration of autoantibodies to desmocollin 1 and clinical review
    British Journal of Dermatology, 2000
    Co-Authors: H Yasuda, T Hashimoto, Hitoshi Kobayashi, K Itoh, M Yamane, J Nakamura
    Abstract:

    We describe a 40-year-old Japanese man with a 3-year history of vesiculoPustular lesions resembling Subcorneal Pustular Dermatosis. Histopathology showed Subcorneal pustules containing a few acantholytic cells, and direct immunofluorescence disclosed IgA deposition in the intercellular space of the upper epidermis. Circulating IgA autoantibodies of very low titre were also demonstrated by indirect immunofluorescence. A novel cDNA transfection technique clearly detected IgA autoantibodies reactive with human desmocollin-1. Combined therapy with dapsone and etretinate improved the skin lesions. We review the clinical features of 49 patients in the literature who presented with vesiculoPustular lesions and intraepidermal IgA deposition.

Jeanjacques Grob - One of the best experts on this subject based on the ideXlab platform.

W Stolz - One of the best experts on this subject based on the ideXlab platform.

  • Rapid response of IgA pemphigus of Subcorneal Pustular Dermatosis type to treatment with isotretinoin.
    Journal of the American Academy of Dermatology, 2020
    Co-Authors: C Gruss, D Zillikens, T Hashimoto, M Amagai, M Kroiss, T Vogt, M Landthaler, W Stolz
    Abstract:

    Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA Dermatosis type and Subcorneal Pustular Dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with Subcorneal Pustular Dermatosis type of IgA pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA pemphigus. Isotretinoin was an effective drug in the treatment of Subcorneal Pustular Dermatosis type of IgA pemphigus in this patient.

  • rapid response of iga pemphigus of Subcorneal Pustular Dermatosis type to treatment with isotretinoin
    Journal of The American Academy of Dermatology, 2000
    Co-Authors: C Gruss, D Zillikens, T Hashimoto, M Amagai, M Kroiss, T Vogt, M Landthaler, W Stolz
    Abstract:

    Abstract Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA Dermatosis type and Subcorneal Pustular Dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with Subcorneal Pustular Dermatosis type of IgA pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA pemphigus. Isotretinoin was an effective drug in the treatment of Subcorneal Pustular Dermatosis type of IgA pemphigus in this patient. (J Am Acad Dermatol 2000;43:923-6.)

J L Mege - One of the best experts on this subject based on the ideXlab platform.