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Related Experiment Videos

Xanthelasmoid mastocytosis.

A Revert1, E Jordá, D Ramón

  • 1Servicio de Dermatologia, Hospital Clínico Universitario, Valencia, Spain.

Pediatric Dermatology
|June 1, 1991
PubMed
Summary
This summary is machine-generated.

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This case study details a rare form of cutaneous mastocytosis, specifically xanthelasmoid mastocytosis, presenting as vulval lesions in a young girl. Treatment with hydroxyzine managed symptoms, with no systemic involvement observed during follow-up.

Area of Science:

  • Dermatology
  • Pediatric Dermatology
  • Histopathology

Background:

  • Mastocytosis is a rare disorder characterized by the abnormal proliferation of mast cells.
  • Cutaneous mastocytosis can present in various forms, including papular, plaque, and diffuse cutaneous types.
  • Xanthelasmoid mastocytosis is a rare subtype with distinctive clinical and histological features.

Observation:

  • An 8-year-old girl presented with a six-year history of small, papular, yellowish vulval lesions exhibiting surface depressions.
  • Clinical observations included symptoms attributed to mast cell mediator release.
  • Histopathological examination revealed a dense, monomorphous infiltrate of rounded cells with granular cytoplasm in the deep dermis.

Findings:

  • The characteristic morphology and red-purple metachromatic staining confirmed the diagnosis of xanthelasmoid mastocytosis.

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  • Symptoms were effectively controlled with hydroxyzine medication.
  • Annual follow-up indicated no evidence of systemic mast cell involvement.

    • Implications:

    • This case highlights the importance of recognizing rare variants of cutaneous mastocytosis in pediatric patients.
    • Hydroxyzine demonstrated efficacy in managing symptoms associated with vulval xanthelasmoid mastocytosis.
    • Surgical intervention may be considered for future management due to the lesion's location.