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Related Concept Videos

Skin Diseases and Disorders01:23

Skin Diseases and Disorders

Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Reticular Dermis

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Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...

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Spatial and Temporal Control of Murine Melanoma Initiation from Mutant Melanocyte Stem Cells
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Neonatal cutaneous mastocytosis.

Somosri Ray1, Asha Mukherjee, Nandita Chatterjee

  • 1Department of Paediatrics, VIMS (RKMSP), Kolkata 700026.

Journal of the Indian Medical Association
|December 23, 2011
PubMed
Summary
This summary is machine-generated.

Neonatal cutaneous mastocytosis, a rare skin disorder, can present with systemic issues even in infants. This case highlights urticaria pigmentosa in a 3 1/2-month-old, emphasizing potential complications and the need for careful monitoring.

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Area of Science:

  • Pediatric Dermatology
  • Hematology
  • Immunology

Background:

  • Neonatal cutaneous mastocytosis is a rare infiltrative skin disorder.
  • While often asymptomatic, systemic involvement can occur at any age.
  • Urticaria pigmentosa is the most common form of cutaneous mastocytosis.

Observation:

  • A 3 1/2-month-old female presented with recurrent tense bullae, flushing, and irritability since day 3 of life.
  • Physical examination revealed Darier sign and dermatographism.
  • Skin biopsy confirmed urticaria pigmentosa.

Findings:

  • The infant exhibited deranged liver function.
  • Treatment included H1 antihistamines and topical glucocorticoids.
  • The family was counseled on anaphylaxis risks and systemic mastocytosis.

Implications:

  • This case underscores the importance of recognizing neonatal cutaneous mastocytosis and its potential systemic manifestations.
  • Early diagnosis and management are crucial for preventing severe complications like anaphylaxis.
  • Long-term monitoring is recommended for infants diagnosed with urticaria pigmentosa.