Linear IgA Disease: A Heterogeneous Disease

Abstract

Data from our series of 70 patients support the hypothesis that linear IgA disease is a heterogeneous disease as regards clinical features, target antigens and immunogenetics. The clinical presentation varies as regards age of onset and severity of skin and mucosal involvement and scarring. The use of split skin and of the novel substrate cylindroma has identified 2 target antigens. The majority of patients have a target antigen associated with epidermal cells and possibly cylindroma hemidesmosomes. A minority have a dermal antigen which resembles collagen VII on cylindroma. There is an association with HLA-B8, -DR3, -Cw7 and with the linked rare tumour necrosis factor a allele, but this is not universal. These differences do not correlate with each other.

Unusual clinical manifestation of linear IgA dermatosis: A report of two cases

“…So one might interpret these patients as representing a new clinical variant with acral lesions reminiscent of erythema gyratum repens, erythema annulare centrifugum, or tinea manuum with histological and immunofluorescence features identical to LAD. In accordance with the observation that circulating antibodies have been only demonstrated in less than 30% of LAD cases, 23,26 in our patients antibodies might as well have been present in concentrations below the sensitivity of detection. 27 In our cases, we did not find an association with malignancy or drug administration, nor with rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis, dermatomyositis, or Crohn's disease, all of which have been described as being associated with LAD.…”

A Case of Linear IgA Disease: An Immunofluorescent Study Using Confocal Laser Scan Microscopy

“…Some patients' sera react with IgA on the epidermal side of 1M NaCl-split skin immunohistologically; others, on the dermal side. Western immunoblot analysis has detected several species of antigen, including 97 kDa protein, in epidermal extracts (7). In the present study, indirect immunofluorescence showed that the patient's serum reacted immunohistologically with the epidermal side of 1M NaCI-split skin.…”

“…Serum titers of CA19-9, a FP, CEA, and ferritin were within normal limits, but the SCCAg concentration was slightly elevated (3.9 ng/ml). HLA typing revealed A (2, 31), B (39,61), C (3,7), and DR (6,8) loci. No internal malignancy was detected by chest X-ray, abdominal X-ray, CT of the trunk, abdominal echography, or gallium scintigraphy.…”

Linear IgA Disease: Cylindroma Demonstrates Heterogeneity of the Target Antigens

“…The localization of the target antigens was studied using NaClsplit skin which splits the BMZ through the lamina lucida allowing differentiation of epidermal from dermal antigen [26].…”