Association of urticaria pigmentosa with café-au-lait spots, neurofibromas and neurofibroma-like neoplasms: a mere coincidence?
BACKGROUND
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 and 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.
Möhrenschlager
Matthias
M
Department of Dermatology and Allergy Biederstein, Technical University of Munich, Germany. m.moehrenschlager@lrz.tum.de
Engst
Reinhard
R
Müller-Weihrich
Stefan
S
Spiessl
Walter
W
Rüdisser
Klaus
K
Weigl
Lorenz B
LB
Jessberger
Berthold
B
Braun-Falco
Markus
M
Abeck
Dietrich
D
Ring
Johannes
J
D000293:Adolescent; D001706:Biopsy; D019080:Cafe-au-Lait Spots / Q000150:complications* / Q000175:diagnosis; D002648:Child; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018318:Neurofibroma, Plexiform / Q000150:complications / Q000175:diagnosis; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D012878:Skin Neoplasms / Q000150:complications* / Q000175:diagnosis; D014582:Urticaria Pigmentosa / Q000150:complications* / Q000175:diagnosis