Patients present with progressive fusiform swelling, erythema, and ten
derness, usually affecting a single digit on the radial side of the ha
nd. A cup-shaped lytic lesion of the distal phalanx is a uniform findi
ng on radiography. Delay in diagnosis and misdiagnosis are common beca
use of the rarity of the lesion and difficulties with histological dif
ferentiation from subungual squamous cell carcinoma. Accurate diagnosi
s requires a high index of suspicion, a careful history, and histologi
cal evaluation. The natural progression of the disease appears to be c
ontinued growth with ongoing destruction of the distal phalanx. Proper
treatment involves surgical removal of the mass by curettage and clos
e follow-up for at least 2 years to monitor for local recurrence.