A middle-aged woman is revealed to have subepithelial bullae with granular IgA deposits on basement membrane and she has some degree of gluten sensitivity. What is the diagnosis?
Ans. c (Dermatitis herpetiformis) (Ref. Robbin'S pathology 8th ed., 484; Harrison's 17th ed., Table 55-1)DERMATITIS HERPETIFORMIS# DH is cha by intensely pruritic, papulovesicular skin lesions symmetrically distributed over extensor surfaces.# All have asso gluten-sensitive enteropathy, and >90% express the HLA-B8/DRw3 and HLA-DQw2 haplotypes.# Biopsy of early lesional = neutrophil-rich infiltrates in dermal papillae. Older lesions = subepidermal bulla.# Studies demonstrate granular deposits of IgA in the papillary dermis and along the epidermal BM zone.# High-avidity IgA autoantibodies against epidermal transglutaminase 3.# Increased incidence of thyroid abnormalities, achlorhydria, atrophic gastritis, and anti gastric parietal cell autoantibodies.# Rx: The mainstay of treatment of DH is dapsone, a sulfone and Gluten free diet.Infectious/Autoimmune Associations with Lymphoma# Gluten enteropathy=# Intestinal T-cell lymphoma# Helicobacter pylori=# Primary gastric malt lymphoma or DLBCL# Chlamydia psittaci=# Ocular adnexal malt lymphoma# Borrelia burgdorferi=# Cutaneous malt lymphoma# Hepatitis C virus=# Splenic marginal zone lymphoma# Campylobacter jejuni=# Immunoproliferative small intestinal disease(IPSID or heavy chain disease)# Sjogren's syndrome=# Extranodal malt lymphoma or DLBCL# Hashimoto's thyroiditis=# Extranodal malt lymphoma or DLBCL
Pemphigus foliaceus
Pemphigus vulgaris
Dermatitis herpetiformis
Bullous pemphigoid
Skin