An infant evaluated for infantile spasms is noted to have several oval hypopigmented patches best seen under Wood's lamp. Which skin lesion of tuberous sclerosis typically appears latest in life?

• A Periungual fibromas (Koenen tumours) ✓
• B Facial angiofibromas
• C Hypomelanotic macules
• D Shagreen patch

Explanation

In tuberous sclerosis, hypomelanotic (ash leaf) macules are present at or soon after birth and are often the first sign, facial angiofibromas and shagreen patches develop in childhood, while periungual Koenen fibromas appear around puberty or later, making them the latest cutaneous feature. Recognising ash leaf macules under Wood's lamp allows early diagnosis before seizures or cardiac rhabdomyomas declare themselves.

Reference: Fitzpatrick's Dermatology, 9th ed.

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