Giant periungual and subungual fibromas (Koenen’s tumor) of tuberous sclerosis complex

A 60-year-old female presented with multiple, asymptomatic papules, nodules, and tumors over the proximal and lateral nail folds and adjoining areas of the toes and fingers since 10–12 years of age. All the fingers and toes except the left second toe showed the lesions [Figures 1 and 2]. The size of the papulonodular lesions and tumors varied between few millimeters and 1–3 cm. Some of the tumors were smooth surfaced and many of them were lobulated and showed minute filiform projections on the surface. She denied a history of seizures and her mental status appeared normal. Physical examination showed multiple, skin colored papules over the nose and nasolabial area. An ill-defined indurated plaque of 15 cm × 10 cm was observed over the right lower back.

Close

Figure 1:

Lobulated tumors and nodules, some of them smooth surfaced and some showing filiform projections involving the proximal and lateral nail folds of all fingers

Export to PPT

Close

Figure 2:

Lobulated tumors and nodules showing minute filiform projections involving proximal and lateral nail folds of all toes, except second left toe

Export to PPT

The unusual and strikingly large, lobulated periungual fibromas involving almost all the fingers and toes, along with typical angiofibromas of the face and Shagreen patch over the right lower back favored the clinical diagnosis of tuberous sclerosis complex.

The patient refused biopsy of the lesions over the toes and fingers.