Dermal eosinophilic infiltrate in junctional epidermolysis bullosa

作者：Saraiya Ami; Yang Catherine S; Kim Jinah; Bercovitch Lionel; Robinson Bostom Leslie; Telang Gladys^*

来源：Journal of Cutaneous Pathology, 2015, 42(8): 559-563.

DOI：10.1111/cup.12521

• basement membrane zone
• bullous diseases
• dermatopathology
• genodermatoses
• histopathology

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 64 and integrin 3. 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.

• 出版日期2015-8