[Cutaneous mastocytosis - update and clinical guidelines]

Area of Science:

• Dermatology
• Hematology
• Rare Diseases

Background:

• Mastocytosis is a rare condition characterized by mast cell accumulation.
• It commonly presents as skin lesions (cutaneous mastocytosis), often diagnosed late.
• Lesions may resolve in children but persist and spread internally in adults.

Purpose of the Study:

• To describe symptoms and signs of cutaneous mastocytosis.
• To provide diagnostic guidelines based on international consensus.
• To present an updated classification of cutaneous mastocytosis forms.

Main Methods:

• Review of international consensus documents.
• Description of clinical presentation (symptoms and signs).
• Inclusion of a new classification system.

Main Results:

• Cutaneous mastocytosis presents with diverse skin lesions, often misdiagnosed.
• Children's lesions may resolve, while adults risk systemic mastocytosis.
• Updated classification categorizes various forms of cutaneous mastocytosis.

Conclusions:

• Early recognition and diagnosis of cutaneous mastocytosis are crucial.
• Adults diagnosed with cutaneous mastocytosis need assessment for systemic disease.
• The updated classification aids in understanding and managing mastocytosis subtypes.