Periungual Fibromas in Tuberous Sclerosis
Periungual fibromas—also called Koenen tumors—are a classic skin manifestation of tuberous sclerosis complex (TSC). Benign, flesh-colored or reddish fibrous growths that arise around or underneath the finger- or toenails (periungual or subungual). They originate from angiofibromas in the nail fold.
Why Do They Occur in Tuberous Sclerosis?
TSC is caused by mutations in TSC1 or TSC2, leading to mTOR hyperactivation and hamartoma formation.
Periungual fibromas are one type of hamartoma characteristic of the condition.
Clinical Features
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Usually appear around puberty or early adulthood
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More common on toenails than fingernails
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Can cause:
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Pain or tenderness
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Nail deformity (longitudinal grooves, splitting)
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Bleeding or catching on clothing
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Typically multiple lesions in TSC, although a single lesion may appear in individuals without TSC.
When to Suspect TSC from Nail Lesions
Consider evaluation for TSC if:
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Multiple periungual fibromas, especially in teens or adults
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Coexisting features like facial angiofibromas, hypopigmented macules (“ash leaf spots”), shagreen patch, cortical tubers, renal angiomyolipomas, epilepsy, or family history of TSC
Management of Periungual Fibromas in Tuberous Sclerosis
– Medical
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Topical sirolimus (mTOR inhibitor) may reduce size or slow progression.
– Procedural
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Shave excision
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Electrocoagulation
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Laser therapy (CO₂ or pulsed dye)
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Surgical excision for deep or broad-based lesions
Recurrence can occur, because the underlying genetic drive remains.
We are independent prescribers able to prescribe any medication from the BNF relevant to the lower limb, foot and ankle.
Are you suffering from any nail condition? At The Chelsea Clinic, we can help. One of our podiatrist can assist and then recommend what treatments are best to get you back on track. Podiatrist South Kensington
Schedule an appointment here or you may call us at +44 (0) 207 101 4000.
We hope you have a feetastic day!
-The Chelsea Clinic and Team
