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Updated: Aug 21, 2026
Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Fibrillar-Type Dermatitis Herpetiformis: An Underrecognized Variant That Is Rarely Associated With Celiac Disease and
Jeffrey Damman1, Elodie Mendels2, Sven van der Woude3
1Department of Pathology, Erasmus Medical Center Rotterdam, Rotterdam, the Netherlands.
Abstract:
Dermatitis herpetiformis (DH) is a rare, intensely pruritic cutaneous manifestation of celiac disease (CD), typically presenting with excoriated papulovesicular lesions on extensor surfaces. DH is associated with IgA autoantibodies against epidermal transglutaminase (eTG), which form granular immune deposits in the papillary dermis and induce subepidermal blistering. Histopathology usually shows papillary dermal microabscesses and granular IgA deposits on direct immunofluorescence. We describe two patients with atypical DH featuring fibrillar, rather than granular, IgA deposits and no evidence of CD. Importantly, in both patients, we are the first to show that eTG did not colocalize with the fibrillar IgA deposits in the skin. Awareness of this pattern is essential to avoid misdiagnosis and potentially unwarranted gluten-free diets, as fibrillar DH is often unrelated to CD.
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