Association of Urticaria pigmentosa with Café-au-Lait Spots, Neurofibromas and Neurofibroma-Like Neoplasms: A Mere Coincidence?

Matthias Möhrenschlager, Reinhard Engst, Stefan Müller-Weihrich, Walter Spiessl, Klaus Rüdisser, Lorenz B. Weigl, Berthold Jessberger, Markus Braun-Falco, Dietrich Abeck, Johannes Ring
2003 Dermatology  
Urticaria pigmentosa (UP) is characterized by dense aggregates of mast cells in the dermis. There is consistent evidence from the literature that mast cells may play a pathogenetic role in the development of neurofibromas and other tumors. Objective: To study the concomitant appearance of UP with neurofibromas and neurofibroma-like neoplasms. Methods: We analyzed 31,752 records of patients examined at the Department of Dermatology in the year 2000, looking for UP and associated neurofibromas
more » ... neurofibroma-like neoplasms in persons younger than 18 years. Results: We identified a total of 27 patients suffering from UP, with 16 persons younger than 18 years. One 12-year-old Caucasian boy demonstrated multiple cutaneous mastocytomas consistent with the diagnosis of UP. On his trunk, four café-au-lait spots were found. A cutaneous neurofibroma was confirmed by skin biopsy. Magnetic resonance imaging detected multiple neoplasms located at the nerve roots of the spine, resembling plexiform neurofibromas. Conclusions: There may be a concomitant appearance of UP and neoplasms, with mast cells possibly playing a causative role. The existence of neoplasms, including neurofibromas and neurofibroma-like lesions should be considered when examining UP cases.
doi:10.1159/000069940 pmid:12771469 fatcat:fphjrmxz7jhk3o5airihjkouru