hereditary progressive mucinous histiocytosis
Go to external page http://purl.obolibrary.org/obo/MONDO_0007725
Hereditary progressive mucinous histiocytosis is 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 middermis. [ Orphanet:158025 ]
Term information
- UMLS:C1840586 (MONDO:equivalentTo)
- Orphanet:158025 (OMIM:142630)
- GARD:16989 (MONDO:GARD)
- OMIM:142630 (Orphanet:158025/e)
- MESH:C564186 (MONDO:equivalentTo)
- MEDGEN:326771 (MONDO:equivalentTo)
gard_rare, ordo_disorder, rare, nord_rare, orphanet_rare
http://identifiers.org/medgen/326771
http://identifiers.org/mesh/C564186
https://omim.org/entry/142630
http://linkedlifedata.com/resource/umls/id/C1840586
http://www.orpha.net/ORDO/Orphanet_158025