[Mastocytosis in children]. / Mastozytose bei Kindern.
Dermatologie (Heidelb)
; 74(5): 323-329, 2023 May.
Article
in De
| MEDLINE
| ID: mdl-37140636
ABSTRACT
Mastocytosis in children is a rare disease characterized by an abnormal accumulation of tissue mast cells. Mastocytosis in children presents with typical skin alterations that are classified as maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis, or mastocytoma. Some patients also develop mast cell mediator symptoms, such as pruritus, flush, and anaphylaxis. In many children, the disease is characterized by a benign and usually self-limiting course; systemic mastocytosis with extracutaneous involvement and a chronic or progressive course is found only rarely. Therapeutically, H1 antihistamines are primarily used on an as-needed basis or as continuous treatment, depending on the severity. Children, parents and caregivers should be thoroughly educated about the clinical picture and possible trigger factors of mast cell mediator release. For children with extensive skin alterations and severe symptoms, the prescription of an epinephrine auto-injector is recommended for emergency treatment.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Mastocytosis
/
Urticaria Pigmentosa
/
Mastocytosis, Cutaneous
/
Mastocytosis, Systemic
Type of study:
Diagnostic_studies
Limits:
Child
/
Humans
Language:
De
Journal:
Dermatologie (Heidelb)
Year:
2023
Document type:
Article