hereditaire progressieve mucineuze histiocytose (aandoening)
hereditaire progressieve mucineuze histiocytose
erfelijke progressieve mucineuze histiocytose
Hereditary progressive mucinous histiocytosis
A rare benign non-Langerhans cell histiocytosis characterized by childhood or adolescence onset of multiple, small, asymptomatic, slowly progressing, skin-colored to red-brown papules with predilection for the face, dorsal hands, forearms and legs, without associated mucosal or visceral involvement. Histologically, papules are well-circumscribed, unencapsulated, nodular aggregates of histiocytes with abundant mucin in the upper and mid dermis.
Clinical courseprogressief
Associated morphologymucineuze histiocytose
Finding sitestructuur van huid
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TermOverige histiocytosesyndromen
SNOMED CT to Orphanet simple map158025
SNOMED CT to ICD-10 extended map
AdviceALWAYS D76.3
CorrelationSNOMED CT source code to target map code correlation not specified