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Hereditary progressive mucinous histiocytosis (disorder)
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.
hereditaire progressieve mucineuze histiocytose
erfelijke progressieve mucineuze histiocytose
Id771300001
StatusPrimitive
Clinical courseProgressive
Associated morphologyMucinous histiocytosis
Finding siteSkin structure
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetD76.3
RuleTRUE
AdviceALWAYS D76.3
CorrelationSNOMED CT source code to target map code correlation not specified