Author(s): Devi B, Dash M, Behera B, Puhan MR
Tuberous sclerosis complex, the preferred name for the condition previously known as tuberous sclerosis, is an autosomal dominant genodermatosis, classically presenting as facial angiofibromas, shagreen patch, periungual fibromas, and ash-leaf white macules. Seizures, mental retardation, retinal hamartomas, subependymal nodules and internal organ hamartomas are common associations. Koenen tumors are periungual or subungual fibromas appearing as reddish to flesh-colored, smooth, soft to firm papules and nodules emerging from nail folds. They are usually 5–10 mm in length, but may be very large. This can be the only clinically evident abnormality.[1,2] In 50% cases of TSC, it appears at an age between 12 and 14 years and increases progressively in size and number with age. The tumors sometimes become hyperkeratotic, resembling fibrokeratoma.[3,4] Histologically, Koenen tumors are characterized by fibrosis with stellate shaped strands of fibroblasts and dense collagen with numerous ectatic blood vessels. Multiple Koenen tumors, affecting all the fingers and toe nails, with a bigger size and morphologically resembling periungual wart, have not been reported earlier.