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Linear IgA disease in adults

J N Leonard, G P Haffenden, N P Ring

    The British Journal of Dermatology
    |September 1, 1982
    PubMed
    Summary
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    This study compared papillary IgA dermatitis herpetiformis (DH) with linear IgA disease subtypes. Findings suggest linear IgA disease is not uniform, necessitating distinct classification for adult linear IgA diseases.

    Area of Science:

    • Dermatology
    • Immunodermatology
    • Gastroenterology

    Background:

    • Differentiating IgA-mediated skin diseases is crucial for accurate diagnosis and treatment.
    • Papillary IgA dermatitis herpetiformis (DH) and linear IgA disease (LAD) share some clinical and histological features, complicating classification.
    • Previous studies have not fully elucidated the distinct characteristics and potential heterogeneity within LAD.

    Purpose of the Study:

    • To compare adult patients with papillary IgA DH, homogeneous-linear IgA (HL) deposits, and granular-linear IgA (GL) deposits.
    • To identify distinct clinical, immunological, and etiological features among these IgA-mediated dermatoses.
    • To propose a refined classification for adult linear IgA diseases.

    Main Methods:

    • A multi-center comparative study involving 35 adult patients with papillary IgA DH and 42 patients with LAD (34 HL, 8 GL).

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  • Assessment included clinical presentation, histology, circulating immune complexes, auto-antibodies, spontaneous remission rates, dapsone response, potassium iodide patch tests, associated enteropathy, and HLA-B8 status.
  • Statistical analysis was performed to compare the three patient groups.

    • Main Results:

    • Groups were similar in age of onset, immune complex/auto-antibody presence, remission rates, histology, and dapsone response.
    • Female predominance in HL group versus male predominance in DH and GL groups.
    • Enteropathy incidence was 85% in DH, 30% in GL, and 24% in HL.
    • HLA-B8 association was highest in DH (88%) compared to GL (50%) and HL (56%).
    • Clinical diagnosis was challenging, especially for the HL group, with presentations resembling DH or pemphigoid.
    • Positive potassium iodide patch tests were more frequent in the DH group.

    Conclusions:

    • Linear IgA deposits may represent a heterogeneous group, not a single entity.
    • Clinical presentation alone is insufficient for diagnosing homogeneous-linear IgA deposits.
    • The term 'adult linear IgA diseases' is proposed to distinguish these patients from those with papillary IgA deposits, pending further subclassification.
    • Associated enteropathy is significantly more common in papillary IgA DH than in linear IgA disease subtypes.