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.
© 2010 Wiley Periodicals, Inc.
MeSH terms
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Blister / diagnosis
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Blister / drug therapy
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Blister / pathology
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Cromolyn Sodium / therapeutic use
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Dermatitis, Atopic / diagnosis
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Dermatitis, Atopic / drug therapy
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Dermatitis, Atopic / pathology
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Dermatologic Agents / therapeutic use
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Exanthema / diagnosis
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Exanthema / drug therapy
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Exanthema / pathology
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Food Hypersensitivity / blood
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Food Hypersensitivity / diet therapy
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Histamine / blood
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Histamine H1 Antagonists / therapeutic use
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Humans
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Immunoglobulin E / blood
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Infant
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Infant, Newborn
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Male
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Mast Cells / drug effects
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Mast Cells / pathology
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Mastocytosis, Cutaneous / diagnosis*
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Mastocytosis, Cutaneous / drug therapy
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Mastocytosis, Cutaneous / pathology
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Proto-Oncogene Proteins c-kit / genetics
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Steroids / therapeutic use
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Tryptases / blood
Substances
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Dermatologic Agents
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Histamine H1 Antagonists
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Steroids
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Immunoglobulin E
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Histamine
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Proto-Oncogene Proteins c-kit
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Tryptases
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Cromolyn Sodium