diffuse cutaneous mastocytosis
Findings
No curated finding names diffuse cutaneous mastocytosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM) and one with infiltrations (Pseudoxanthomatous DCM).
Definition from the Mondo Disease Ontology (MONDO:0019315), read 2026-09-29. CC BY 4.0.
Features
31 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.
- Darier's signHPOHP:0025081
- Very frequent (80% to 99% of cases)
- Elevated total serum tryptaseHPOHP:0031901
- Very frequent (80% to 99% of cases)
- FlushingHPOHP:0031284
- Very frequent (80% to 99% of cases)
- Generalized abnormality of skinHPOHP:0011354
- Very frequent (80% to 99% of cases)
- PruritusHPOHP:0000989
- Very frequent (80% to 99% of cases)
- Abnormal blistering of the skinHPOHP:0008066
- Frequent (30% to 79% of cases)
Show the remaining 19
- Peau d'orangeHPOHP:0025533
- Frequent (30% to 79% of cases)
- Skin erosionHPOHP:0200041
- Frequent (30% to 79% of cases)
- Thickened skinHPOHP:0001072
- Frequent (30% to 79% of cases)
- UrticariaHPOHP:0001025
- Frequent (30% to 79% of cases)
- VomitingHPOHP:0002013
- Frequent (30% to 79% of cases)
- Abdominal painHPOHP:0002027
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: diffuse cutaneous mastocytosis
- Also called
- diffuse cutaneous maculopapulous mastocytosis