Mastocytosis is diagnosed without difficulty if it presents with easily rec
ognizable lesions of urticaria pigmentosa. Recently, we have identified har
dly visible skin lesions of mastocytosis in Hymenoptera venom allergic pati
ents ("occult mastocytosis"). In addition, in approximately 15% of the pati
ents with typical cutaneous lesions, urticaria pigmentosa was at first mist
aken for other conditions and thus not linked to simultaneous symptoms of s
ystem ic mastocytosis. In most patients with unrecognized mastocytosis,the
diagnosis was supported by raised basal serum tryptase levels. Cutaneous ma
stocytosis is often overlooked and more frequent than assumed. Measurement
of basal serum tryptase concentrations can make an important contribution t
o the diagnosis of mastocytosis, but it does not replace a meticulous skin
examination.