Mastocytosis
Melody C Carter, Dean D Metcalfe, Hirsh D Komarow, Melody C Carter, Dean D Metcalfe, Hirsh D Komarow
Abstract
Mastocytosis is a disorder of abnormal mast cell proliferation, with clinical features that include flushing, pruritus, abdominal pain, diarrhea, hypotension, syncope, and musculoskeletal pain. These features are the result of mast cell mediator release and infiltration into target organs. Patients of all ages may be affected, although in children, manifestations primarily involve the skin. Most patients with systemic disease have a somatically acquired activating mutation in the KIT oncogene. This article discusses the causes and pathogenesis of mastocytosis, with an overview of the clinical features and the approach to diagnosis, evaluation, and therapy in adults and pediatric patients.
Keywords: Bone marrow; KIT; Mast cell; Mastocytosis; Skin; Urticaria.
Conflict of interest statement
Authors state no conflict of interest.
Published by Elsevier Inc.
Figures
![Figure 1. Urticaria Pigmentosa in an adult](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f1.jpg)
Figure 2. Urticaria pigmentosa in a child
Figure 2. Urticaria pigmentosa in a child
Tan papules and thin plaques on this child’s…
Figure 3. Diffuse cutaneous mastocytosis
A) Diffuse…
Figure 3. Diffuse cutaneous mastocytosis
A) Diffuse dermal thickening, pachydermic flesh colored lesions and B)…
Figure 3. Diffuse cutaneous mastocytosis
A) Diffuse…
Figure 3. Diffuse cutaneous mastocytosis
A) Diffuse dermal thickening, pachydermic flesh colored lesions and B)…
Figure 4. Solitary mastocytoma on the foot…
Figure 4. Solitary mastocytoma on the foot sole of a child
A flat red to…
Figure 5. Histopathology of Urticaria Pigmentosa from…
Figure 5. Histopathology of Urticaria Pigmentosa from a 7 yr old
Clustering of normal and…
Figure 6. Bone marrow histopathology in an…
Figure 6. Bone marrow histopathology in an adult with Indolent Systemic Mastocytosis
Mast cell aggregates…
![Figure 2. Urticaria pigmentosa in a child](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f2.jpg)
![Figure 3. Diffuse cutaneous mastocytosis](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f3a.jpg)
![Figure 3. Diffuse cutaneous mastocytosis](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f3b.jpg)
![Figure 4. Solitary mastocytoma on the foot…](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f4.jpg)
![Figure 5. Histopathology of Urticaria Pigmentosa from…](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f5.jpg)
![Figure 6. Bone marrow histopathology in an…](https://www.ncbi.nlm.nih.gov/pmc/articles/instance/3863935/bin/nihms531191f6.jpg)
Source: PubMed