Langerhans cell histiocytosis
MONDO:0018310Mondo
Findings
No curated finding names Langerhans cell histiocytosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Langerhans cell histiocytosis (LCH) is a systemic disease associated with the proliferation and accumulation (usually in granulomas) of Langerhans cells in various tissues.
Definition from the Mondo Disease Ontology (MONDO:0018310), read 2026-09-29. CC BY 4.0.
Features
27 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Bone painHPOHP:0002653
- Very frequent (80% to 99% of cases)
- OsteolysisHPOHP:0002797
- Very frequent (80% to 99% of cases)
- CoughHPOHP:0012735
- Frequent (30% to 79% of cases)
- Eczematoid dermatitisHPOHP:0000964
- Frequent (30% to 79% of cases)
- FeverHPOHP:0001945
- Frequent (30% to 79% of cases)
- HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
- LymphadenopathyHPOHP:0002716
- Frequent (30% to 79% of cases)
- Skin rashHPOHP:0000988
- Frequent (30% to 79% of cases)
- SplenomegalyHPOHP:0001744
- Frequent (30% to 79% of cases)
- AtaxiaHPOHP:0001251
- Occasional (5% to 29% of cases)
- Decreased total leukocyte countHPOHP:0001882
- Occasional (5% to 29% of cases)
- Diabetes insipidusHPOHP:0000873
- Occasional (5% to 29% of cases)
Show the remaining 15
- DysarthriaHPOHP:0001260
- Occasional (5% to 29% of cases)
- Genital ulcersHPOHP:0003249
- Occasional (5% to 29% of cases)
- Growth delayHPOHP:0001510
- Occasional (5% to 29% of cases)
- Hearing impairmentHPOHP:0000365
- Occasional (5% to 29% of cases)
- HypoalbuminemiaHPOHP:0003073
- Occasional (5% to 29% of cases)
- Mental deteriorationHPOHP:0001268
- Occasional (5% to 29% of cases)
Where it sits
- Narrower terms (8)
- eosinophilic granuloma
- hand-Schuller-Christian disease
- Langerhans cell histiocytosis specific to adulthood
- Langerhans cell histiocytosis specific to childhood
- multisystem langerhans cell histiocytosis
- pulmonary langerhans cell histiocytosis
- single-system multifocal langerhans cell histiocytosis
- unifocal langerhans cell histiocytosis
Other names
5 names
Resolves to: Langerhans cell histiocytosis
- Also called
- histiocytosis XLangerhans cell granulomatosisLangerhans cell histiocytosis, NOSLangerhans cell histiocytosis, Not otherwise specifiedLCH