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Syringocystadenoma papilliferum

Disease definition

A rare non-malignant adnexal neoplasm that originates from the apocrine or eccrine sweat glands and is characterized histologically by cystic, papillary, and ductal invaginations into the dermis lined by double-layered outer cuboidal and luminal high columnar epithelium and connected to the epidermis. Dilated capillaries and a dense infiltrate of plasma cells are characteristic. Clinically, lesions are asymptomatic with a heterogeneous, non-distinctive appearance ranging from skin-colored to pink papules or plaques, occurring most commonly in the head and neck area.

ORPHA:840

Classification level: Disorder
  • Synonym(s):
    • Fistulous vegetative verrucous hydradenoma
    • Naevus syringocystadenomatosus papilliferus
    • Papillary syringocystadenoma
    • SCAP
    • Syringadenoma papilliferum
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood, Neonatal, Infancy, Adolescent
  • ICD-10: D23.9
  • OMIM: -
  • UMLS: C0406803
  • MeSH: -
  • GARD: 5100
  • MedDRA: 10042926
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