Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients

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.