Multiple Koenen Tumors, a Rare Entity: Combination Treatment with 1% Topical Sirolimus Electrofulguration and Excision

Abstract

<b><i>Introduction:</i></b> Koenen tumors are benign, cutaneous manifestations of tuberous sclerosis. These are disfiguring, painful, and challenging to treat as they frequently recur. We report a case of long-standing, multiple Koenen tumors affecting all twenty nails in an elderly female who was successfully treated with a combination of topical sirolimus 1%, surgical excision, and electrofulguration. <b><i>Case Report:</i></b> A 57-year-old lady presented with multiple, asymptomatic periungual, and subungual tumors affecting all twenty nails since 27 years. Cutaneous examination revealed confetti macules, ash-leaf macule, and shagreen patch over trunk. Nail biopsy was compatible with Koenen’s tumor. Computerized tomography of brain showed diffuse patchy sclerosis. The tumors were treated with topical sirolimus 1% ointment for 10 months with excellent regression. Electro­fulguration for both great toenails and surgical excision of right thumbnail periungual fibroma was done. 1% sirolimus was advised after the surgical treatment. There were no adverse effects or recurrence of tumors over a 2-year follow-up. <b><i>Discussion:</i></b> Topical sirolimus 1% was effective in tumor regression and preventing new tumor formation. Larger tumors that interfered in daily chores were treated with excision and electrofulguration. Thus, a combination treatment for this rare presentation of tuberous sclerosis provided optimum results.

Giant Koenen Tumors as the Presenting and Only Clinical Sign of Tuberous Sclerosis Complex in a 56-Year-Old Man and Its Surgical Management

“…These periungual or subungual fibromas appear as reddish to flesh-colored, smooth, and soft to firm papules and nodules typically emerging from the proximal nail fold of the toenails or, less commonly, the fingernails [4, 12]. They are generally 5 to 10 mm in length but may progressively increase in size and form large confluent masses with partial or total destruction of the nail plate [4, 8, 9, 12]. Substantial involvement of the nail apparatus may lead to local pain, recurrent infections, and difficulty to perform daily activities as seen in the presented case.…”

“…In aggressive and/or recurrent cases with high morbidity, some authors recommend surgical extirpation of the entire nail apparatus followed by reconstruction with full-thickness skin graft [8, 21]. Recently, topical sirolimus 1% has proven to be useful in regressing tumor size before surgical intervention in order to reduce recurrences [9]. In the authors experience, complete surgical excision up to the proximal base of the tumor offers satisfactory results in terms of functionally and esthetic appearance, as seen in the presented case.…”

“…Although KTs are usually asymptomatic, they can represent a concern to patients due to nail deformity and associated pain with progressive growth. Treatment is considered a true challenge because recurrences rates are high, and no consensus exists about a standard therapy protocol [7-9].…”