Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature

Pediatr Dermatol. 2011 Sep-Oct;28(5):542-6. doi: 10.1111/j.1525-1470.2010.01212.x. Epub 2010 Jul 29.

Abstract

Diffuse cutaneous mastocytosis is a rare variant of mast cell disease with widespread erythroderma, which is normally clinically apparent in early infancy. We report the case of a neonate who presented with diffuse erythrodermic rash and bullous lesions. Biopsy specimens showed a dense dermal infiltrate of mast cells. Serum histamine and tryptase levels were elevated. No somatic mutation of the c-kit gene was found. Blistering ceased at 5 months of age, but atopic dermatitis appeared at 6 months and allergic workup revealed a high level of food-specific IgE. Herein, we describe the case and provide the first review of the literature on neonatal onset diffuse cutaneous mastocytosis to clarify the prognosis of this condition.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Blister / diagnosis
  • Blister / drug therapy
  • Blister / pathology
  • Cromolyn Sodium / therapeutic use
  • Dermatitis, Atopic / diagnosis
  • Dermatitis, Atopic / drug therapy
  • Dermatitis, Atopic / pathology
  • Dermatologic Agents / therapeutic use
  • Exanthema / diagnosis
  • Exanthema / drug therapy
  • Exanthema / pathology
  • Food Hypersensitivity / blood
  • Food Hypersensitivity / diet therapy
  • Histamine / blood
  • Histamine H1 Antagonists / therapeutic use
  • Humans
  • Immunoglobulin E / blood
  • Infant
  • Infant, Newborn
  • Male
  • Mast Cells / drug effects
  • Mast Cells / pathology
  • Mastocytosis, Cutaneous / diagnosis*
  • Mastocytosis, Cutaneous / drug therapy
  • Mastocytosis, Cutaneous / pathology
  • Proto-Oncogene Proteins c-kit / genetics
  • Steroids / therapeutic use
  • Tryptases / blood

Substances

  • Dermatologic Agents
  • Histamine H1 Antagonists
  • Steroids
  • Immunoglobulin E
  • Histamine
  • Proto-Oncogene Proteins c-kit
  • Tryptases
  • Cromolyn Sodium