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?
Pemphigus foliaceus
Pemphigus vulgaris
Dermatitis herpetiformis
Bullous pemphigoid
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
Skin