Epidermal Transglutaminase Deposits in Perilesional and Uninvolved Skin in Patients with Dermatitis Herpetiformis

Abstract

Brown recluse spider (Loxosceles reclusa) venom phospholipase D (PLD) generates lysophosphatidic acid (LPA).

Dermatitis Herpetiformis Sera or Goat Anti–Transglutaminase-3 Transferred to Human Skin-Grafted Mice Mimics Dermatitis Herpetiformis Immunopathology

“…They also found IgA Abs to epidermal transglutaminase in the serum of DH patients as well as celiac patients without DH. We subsequently confirmed both of these findings using new reagents created in our laboratory (7).…”

“…This is similar to the situation in clinically normal appearing skin near DH lesions where abundant IgA and TG3 are deposited, but there is no inflammation (2,7). It is clear that additional proinflammatory events are required for the eventual development of inflammatory skin lesions.…”

Transglutaminases and Disease: Lessons From Genetically Engineered Mouse Models and Inherited Disorders

“…Common manifestations include enteropathy (celiac disease or celiac sprue) that results from T cell-mediated flattening of the small intestinal mucosa and is characterized by malabsorption and chronic diarrhea; dermatopathy (dermatitis herpetiformis), a blistering skin disease of the extensor surfaces of the major joints; and neuropathy (gluten axonal neuropathy) as well as cerebellar ataxia due to the loss of Purkinje cells. Patients develop IgA antibodies against gluten epitopes and predominantly IgA autoantibodies against TG2 in celiac disease (76), against TG3 in dermatitis herpetiformis (79,227,242), or against TG6 in gluten neuropathy (106). Furthermore, tissue analyses show small intestinal IgA-TG2 deposits in celiac disease (142), dermal IgA-TG3 deposits in dermatitis herpetiformis (242), and cerebellar IgA-TG6 deposits in gluten ataxia (106).…”

Autoantibodies against epidermal transglutaminase are a sensitive diagnostic marker in patients with dermatitis herpetiformis on a normal or gluten-free diet

“…In contrast, eTG-specific antibodies noncross-reactive with tTG are found only in patients with DH. 8 Furthermore, eTG but not tTG colocalizes with granular IgA deposits in the skin of patients with DH 8,9 and levels of antibodies against eTG correlate with the extent of enteropathy in DH but not in GSE without DH. 10 Taken together, these data suggest that eTG rather than tTG is the autoantigenic target in patients with DH.…”