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[Diffuse cutaneous mastocytosis without permanent lesion]

V Legrain1, A Taïeb, P Bioulac-Sage

  • 1Unité de Dermatologie pédiatrique, Hôpital Pellegrin, Bordeaux.

Annales De Dermatologie Et De Venereologie
|January 1, 1994
PubMed
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This study details an infant with cutaneous mastocytosis presenting only with functional symptoms like itching and hives, not typical skin lesions. Dermal mast cell quantification is crucial for diagnosis, distinguishing it from idiopathic anaphylaxis.

Area of Science:

  • Pediatric Dermatology
  • Allergy and Immunology
  • Histopathology

Background:

  • Cutaneous mastocytosis typically presents with visible skin changes like maculopapules or infiltration.
  • Systemic involvement is a common concern in pediatric mastocytosis cases.

Observation:

  • A 9-month-old infant exhibited cutaneous mastocytosis solely through functional symptoms (pruritus, urticaria) without persistent skin lesions.
  • Skin biopsy revealed a 22-fold increase in mastocytes compared to healthy controls, consistent with mastocytosis.

Findings:

  • This case represents an exceptionally rare form of diffuse cutaneous mastocytosis characterized by the absence of permanent skin lesions.
  • The diagnostic threshold for mastocytosis was met based on elevated dermal mastocyte counts.

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Implications:

  • This presentation highlights the importance of considering mastocytosis even in the absence of classic dermatological signs.
  • Quantifying dermal mastocytes is essential to differentiate this condition from idiopathic anaphylaxis.
  • The long-term prognosis for this specific subtype of cutaneous mastocytosis remains undetermined.