Cutaneous mastocytosis: Demographic aspects and clinical features of 55 patients

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. © 2006 European Academy of Dermatology and Venereology.