We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Association of urticaria pigmentosa with café-au-lait spots, neurofibromas and neurofibroma-like neoplasms: a mere coincidence?
- Authors
Möhrenschlager, Matthias; Engst, Reinhard; Müller-Weihrich, Stefan; Spiessl, Walter; Rüdisser, Klaus; Weigl, Lorenz B; Jessberger, Berthold; Braun-Falco, Markus; Abeck, Dietrich; Ring, Johannes
- Abstract
<bold>Background: </bold>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.<bold>Objective: </bold>To study the concomitant appearance of UP with neurofibromas and neurofibroma-like neoplasms.<bold>Methods: </bold>We analyzed 31,752 records of patients examined at the Department of Dermatology in the year 2000, looking for UP and associated neurofibromas and neurofibroma-like neoplasms in persons younger than 18 years.<bold>Results: </bold>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.<bold>Conclusions: </bold>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.
- Subjects
BIOPSY; MAGNETIC resonance imaging; NEUROFIBROMA; SKIN tumors; URTICARIA pigmentosa; CAFE-au-Lait spots (Disease); DISEASE complications; NEUROFIBROMATOSIS 1; DIAGNOSIS
- Publication
Dermatology (10188665), 2003, Vol 206, Issue 4, p297
- ISSN
1018-8665
- Publication type
journal article