Preferred Label : Parotid salivary glands, polycystic dysgenetic disease of;
Symbol : PDDP;
CISMeF acronym : PDDP;
Type : Phenotype or locus, molecular basis unknown;
Description : Polycystic dysgenetic disease of the parotid gland (PDDP) is a rare benign condition
of the parotid gland. The disorder presents often in childhood or young adulthood,
but may occur later in life. It occurs most commonly in females. Features include
fluctuating and nontender swelling of the parotid gland bilaterally, without defects
in salivary function. Histology shows replacement of the lobular portion of the parotid
gland by multiple epithelial-lined cysts arising from the intercalated ducts. The
cysts often contain altered salivary secretions, including spheroliths or microliths;
eosinophilic congophilic deposits have also been described. Chronic inflammation is
not present. The condition is thought to result from a developmental defect of the
intercalated duct system. Surgery may be indicated for diagnosis or for cosmetic reasons
(summary by Smyth et al., 1993; Layfield and Gopez, 2002; Eley et al., 2011).;
Inheritance : Autosomal dominant;
Prefixed ID : %600343;
Origin ID : 600343;
UMLS CUI : C3551133;
CISMeF manual mappings
- HPO term(s)
- Semantic type(s)
