Pilomatrixoma is the only skin appendage tumour showing predominantly
hair matrix differentiation. Rarely, aggressive or malignant variants
are reported in the literature. We report a case of a 44-year-old male
with multiple pilomatrixomas, one of which grew rapidly, recurring se
veral times. Histological examination of this tumour showed predominan
tly hair matrix differentiation but also abortive hair follicle format
ion with pilar keratinization reminiscent of a trichoepithelioma. Nume
rous mitoses and moderate cellular pleomorphism were present, associat
ed with infiltration of subcutaneous skeletal muscle and vascular inva
sion.