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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.
Id771300001
StatusPrimitive
Clinical courseprogressief
Associated morphologymucineuze histiocytose
Finding sitestructuur van huid
DHD Diagnosethesaurus-referentieset
referentieset met complexe 'mapping' naar ICD-10
TargetD76.3
RuleTRUE
AdviceALWAYS D76.3
CorrelationSNOMED CT source code to target map code correlation not specified