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Syringocystadenoma papilliferum

ORPHA:840· ICD-10 D23.9

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.

Prevalence
<1 / 1 000 000
Inheritance
Unknown
Age of onset
Adolescent, Childhood, Infancy, Neonatal