Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients

J Eur Acad Dermatol Venereol. 2006 Sep;20(8):969-73. doi: 10.1111/j.1468-3083.2006.01696.x.

Abstract

Background: Mastocytosis is a rare, heterogeneous group of disorder with abnormal increase of mast cells in one or more organ systems.

Objective: To evaluate the demographic and clinical features of cutaneous mastocytosis (CM).

Methods: Records of 55 patients with cutaneous mastocytosis were retrospectively analysed.

Results: Of the 22 females and 33 males, 80% had urticaria pigmentosa/maculopapular CM and 20% had mastocytoma. Of all cases, 81.8% had first lesions in childhood. The most common presentation was involvement of trunk together with extremities. Thirteen (23.6%) patients had history of bulla; Darier's sign was positive in 34 of 38 patients. Itching was the most common complaint, provocated by hot weather/bath.

Conclusion: Clinical presentations of urticaria pigmentosa/maculopapular CM and mastocytoma are similar regarding gender, age of onset, age of diagnosis, and presence of Darier's sign and history of bulla. In contrast to mastocytoma, urticaria pigmentosa/maculopapular CM lesions were frequently located on trunk together with extremities.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Mastocytosis, Cutaneous / classification
  • Mastocytosis, Cutaneous / epidemiology*
  • Mastocytosis, Cutaneous / physiopathology
  • Middle Aged
  • Retrospective Studies