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[Urticaria pigmentosa: a current approach].

Antonio David Pérez-Elizondo1, Benjamín Zepeda-Ortega, Gladys Teresa del Pino-Rojas

  • 1Servicio de Dermatología, Hospital para el Niño IMIEM, Toluca, Estado de México. apederma@yahoo.com.mx

Revista Alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)
|September 23, 2009
PubMed
Summary

Urticaria pigmentosa (UP) involves abnormal mast cell (MC) growth in the skin, causing symptoms through mediator release or infiltration. This review covers UP symptoms, classification, pathophysiology, and treatments.

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Area of Science:

  • Dermatology
  • Hematology
  • Oncology

Background:

  • Urticaria pigmentosa (UP) is a disorder of abnormal mast cell (MC) proliferation in the skin.
  • Symptoms arise from MC mediator release or neoplastic MC infiltration.
  • Somatic c-kit D816V mutations are frequently implicated, particularly in systemic forms.

Purpose of the Study:

  • To provide an updated overview of urticaria pigmentosa.
  • To enhance understanding of UP symptoms, classification, pathophysiology, and treatment.

Main Methods:

  • Literature review of current research on urticaria pigmentosa.
  • Synthesis of information on molecular, genetic, and chromosomal defects.
  • Analysis of recent advances in pathophysiology and therapeutic strategies.

Main Results:

  • Urticaria pigmentosa presents heterogeneously with diverse clinical manifestations.
  • Pathophysiology involves mast cell dysregulation, often linked to c-kit mutations.
  • Current treatment focuses on managing symptoms and underlying disease processes.

Conclusions:

  • A comprehensive understanding of urticaria pigmentosa requires integrating knowledge of its varied symptoms, classification, and underlying molecular mechanisms.
  • Advances in pathophysiology are paving the way for targeted therapeutic approaches.
  • Further research is crucial for optimizing the management of urticaria pigmentosa.