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[Systemic mastocytosis: clinical case]

P Mardones1, C Moyano, K Peña

  • 1Clínica Miguel de Servet, Departamento de Anatomía Patológica Hospital San Borja-Arriarán, Providencia, Santiago.

Revista Medica De Chile
|November 27, 1998
PubMed
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This case study details a patient with a rare condition causing skin lesions and systemic symptoms like tachycardia and flushing, triggered by medications. Diagnosis involved confirming mast cell infiltration, offering partial symptom relief with specific blockers and stabilizers.

Area of Science:

  • Dermatology
  • Hematology
  • Clinical Medicine

Background:

  • Presents a case of a 38-year-old female with a lifelong history of distinct, dark brown cutaneous lesions.
  • Lesions exhibit inflammatory responses (erythema, urticaria) upon physical stimulation or medication intake.

Observation:

  • The patient experienced recurrent systemic episodes including tachycardia, flushing, headache, abdominal pain, arthralgia, diarrhea, and vomiting.
  • Hospitalizations were necessitated by severe tachycardia, hypotension, generalized urticaria, and altered consciousness, often linked to anti-inflammatory drug or aspirin ingestion.

Findings:

  • Skin and bone marrow biopsies confirmed significant mast cell infiltration.
  • Bone scintiscan also indicated mast cell involvement.

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Implications:

  • The findings suggest a diagnosis of systemic mastocytosis or a related mast cell activation disorder.
  • While H1/H2 blockers and mast cell stabilizers provided partial relief, the case highlights the complex management of such conditions.