Autoimmune acrosyringitis with ductal cysts: reclassification of case of eruptive syringoma.
Syringomas are architecturally complex tumors composed of small, cystically dilated segments of dermal eccrine duct. Syringomas typically form isolated flesh-colored periorbital papules, however, in a peculiar condition termed eruptive syringoma; scores develop simultaneously in near confluence over a large surface area. While traditionally regarded as a neoplasm, more recent observations indicate eruptive syringoma is a reactive proliferation secondary to autoimmune disruption of the acrosyringium. We present the case of a 44-year-old woman with eruptive syringoma of the labia majora and prominent lymphocytic inflammation in the acrosyringium. Immunohistochemical stains confirm that the infiltrate is composed of CD4+ and CD8+ T cells, without significant CD20 B cells or CD138 plasma cells. Sequential sections of the syringoma reveal a complex 3-dimensional architecture with functionally isolated cysts, not connected to adjacent cysts or ducts by a discernable epithelium. These findings support the conclusion that eruptive syringoma is a tortuous proliferation of dermal eccrine ducts and fibrous stroma secondary to autoimmune destruction of the acrosyringium. Conceptually, the disorganized expansion of an eccrine duct syringoma may be analogous to a peripheral nerve traumatic neuroma.
Chandler
Wells M
WM
Pathology, University of Utah Health Sciences, Department of Pathology, Division of Anatomic Pathology, Huntsman Cancer Hospital, Room 3860, Salt Lake City, Utah 84112, USA. Wells.chandler@hsc.utah.edu
Bosenberg
Marcus W
MW
D000328:Adult; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D003560:Cysts / Q000473:pathology; D004439:Eccrine Glands / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007249:Inflammation / Q000473:pathology; D013544:Sweat Gland Neoplasms / Q000276:immunology / Q000473:pathology*; D018252:Syringoma / Q000276:immunology / Q000473:pathology*