Bullous mastocytosis: report of a patient and a brief review of the literature

Am J Dermatopathol. 2008 Oct;30(5):455-7. doi: 10.1097/DAD.0b013e3181783354.

Abstract

We report a 6-month-old male infant who presented to the pediatric dermatology clinic at Wake Forest University Baptist Medical Center with a generalized bullous eruption since 3 months of age. A sepsis work up was performed at an outside hospital before presentation and did not reveal any evidence of systemic infection. Clinical presentation revealed a well-nourished, appropriate-for-age, 6-month-old boy with multiple tense bullae, some in a "string of pearls" arrangement, on the bilateral upper extremities and trunk. Multiple erosions were also noted. Laboratory evaluation revealed a normal complete blood count. Polymerase chain reaction was negative for herpes simplex virus types I and II. Histologic sections demonstrated a large space of separation between the epidermis and dermis which was filled by a monomorphous infiltrate composed of round to oval cells with centrally placed nuclei, consistent with mast cells. Leder and C-Kit stains were strongly positive, confirming the diagnosis of bullous mastocytosis. Treatment included fluocinonide 0.05% cream and tacrolimus 0.1% ointment to active lesions and silver sulfadiazine 1% cream to erosions. Improvement was noted during follow-up examination.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anti-Infective Agents, Local / therapeutic use
  • Fluocinonide / therapeutic use
  • Glucocorticoids / therapeutic use
  • Humans
  • Infant
  • Male
  • Mast Cells / metabolism
  • Mast Cells / pathology
  • Mastocytosis, Cutaneous / diagnosis*
  • Mastocytosis, Cutaneous / drug therapy
  • Mastocytosis, Cutaneous / pathology*
  • Proto-Oncogene Proteins c-kit / metabolism
  • Silver Sulfadiazine / therapeutic use

Substances

  • Anti-Infective Agents, Local
  • Glucocorticoids
  • Fluocinonide
  • Proto-Oncogene Proteins c-kit
  • Silver Sulfadiazine