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Mucinous histiocytosis

MedGen UID:
569560
Concept ID:
C0334126
Disease or Syndrome
Synonym: Histiocytosis, mucinous
SNOMED CT: Mucinous histiocytosis (87412005)
 
HPO: HP:0040138

Definition

Multiple subcutaneous non-fragile and skin-coloured papules characterized by interstitial infiltrate of spindle and epithelioid histiocytes, and mucin. There are well circumscribed aggregates of epithelioid histiocytes and mucin in the upper and middle dermis,with the histiocytes arranged between collagen bundles and separated from the epidermis by a Grenz zone. [from HPO]

Term Hierarchy

Conditions with this feature

Hereditary progressive mucinous histiocytosis
MedGen UID:
326771
Concept ID:
C1840586
Disease or Syndrome
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.

Recent clinical studies

Etiology

Fraitag S, Emile JF
Histopathology 2022 Jan;80(1):196-215. doi: 10.1111/his.14569. PMID: 34958507

Diagnosis

Requena C, Requena L, Traves V, Sanmartín O
J Cutan Pathol 2017 Sep;44(9):781-785. Epub 2017 Jun 19 doi: 10.1111/cup.12970. PMID: 28556021
Nguyen NV, Prok L, Burgos A, Bruckner AL
Pediatr Dermatol 2015 Nov-Dec;32(6):e273-6. Epub 2015 Oct 13 doi: 10.1111/pde.12694. PMID: 26461050
Narváez-Rosales V, Sáez-de-Ocariz M, Toussaint-Caire S, Ortiz-Hidalgo C, Espinosa-Rosales F
Skinmed 2013 May-Jun;11(3):175-8. PMID: 23930359
Hemmati I, McLeod WA, Crawford RI
J Cutan Med Surg 2010 Sep-Oct;14(5):245-8. doi: 10.2310/7750.2010.09047. PMID: 20868622
Young A, Olivere J, Yoo S, Martins C, Barrett T
J Cutan Pathol 2006 Feb;33(2):166-70. doi: 10.1111/j.0303-6987.2006.00274.x. PMID: 16420313

Therapy

Diab R, Shahidi Dadras M, Rakhshan A, Kaddah A, Abdollahimajd F
J Dermatolog Treat 2023 Dec;34(1):2117538. Epub 2022 Oct 21 doi: 10.1080/09546634.2022.2117538. PMID: 36036585

Prognosis

Bork K, Hoede N
Arch Dermatol 1988 Aug;124(8):1225-9. PMID: 3401026

Clinical prediction guides

Young A, Olivere J, Yoo S, Martins C, Barrett T
J Cutan Pathol 2006 Feb;33(2):166-70. doi: 10.1111/j.0303-6987.2006.00274.x. PMID: 16420313
Bork K
Arch Dermatol 1994 Oct;130(10):1300-4. doi: 10.1001/archderm.130.10.1300. PMID: 7944513

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