title: Dermal eosinophilic infiltrate in junctional epidermolysis bullosa.
abstract: Junctional epidermolysis bullosa (JEB) is a rare genodermatosis characterized by a split in the lamina lucida usually because of mutations in LAMA3, LAMB3 and LAMC2 resulting in absence or reduction of laminin-332. Rare subtypes of JEB have mutations in COL17A1, ITGB4, ITGA6 and ITGA3 leading to reduction or dysfunction of collagen XVII, integrin α6β4 and integrin α3. The classic finding under light microscopy is a paucicellular, subepidermal split. We describe the unusual presence of an eosinophilic infiltrate in the bullae and subjacent dermis in a neonate with JEB, generalized intermediate (formerly known as non-Herlitz-type JEB), discuss the histologic differential diagnosis for a subepidermal blister in a neonate, review the literature regarding cases of epidermolysis bullosa (EB) presenting with inflammatory infiltrates, and discuss mechanisms to explain these findings. This case highlights that eosinophils can rarely be seen in EB and should not mislead the dermatopathologist into diagnosing an autoimmune blistering disorder.
authors.affiliation: Tufts Dermatology Research Department, Tufts Medical Center, Boston, MA, USA.
authors.forename: Ami
authors.initials: A
authors.lastname: Saraiya
authors.affiliation: Department of Dermatology, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, RI, USA.
authors.forename: Catherine S
authors.initials: CS
authors.lastname: Yang
authors.affiliation: Department of Pathology, Division of Dermatopathology, Stanford University, Palo Alto, CA, USA.
authors.forename: Jinah
authors.initials: J
authors.lastname: Kim
authors.affiliation: Department of Dermatology, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, RI, USA.
authors.forename: Lionel
authors.initials: L
authors.lastname: Bercovitch
authors.affiliation: Department of Dermatology, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, RI, USA.
authors.forename: Leslie
authors.initials: L
authors.lastname: Robinson-Bostom
authors.affiliation: Department of Dermatology, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, RI, USA.
authors.forename: Gladys
authors.initials: G
authors.lastname: Telang
mesh_terms: D001324:Autoantigens / Q000378:metabolism; D001485:Basement Membrane / Q000473:pathology; D004802:Eosinophilia / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016109:Epidermolysis Bullosa, Junctional / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005455:Fluorescent Antibody Technique; D006801:Humans; D007231:Infant, Newborn; D007797:Laminin / Q000235:genetics / Q000378:metabolism; D008297:Male; D008854:Microscopy, Electron / Q000379:methods; D009154:Mutation; D024041:Non-Fibrillar Collagens / Q000378:metabolism; D000097805:Collagen Type XVII
chemical_list: D001324:Autoantigens; D007797:Laminin; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII
keywords: basement membrane zone; bullous diseases; dermatopathology; genodermatoses; histopathology