A young woman had acral blistering after minor friction during infancy, which improved markedly with age. She now has progressive poikiloderma with atrophy, telangiectasia and mottled pigmentation on the hands and face, plus nail dystrophy. Light microscopy of a fresh blister would show:
- A Cleavage exclusively within the basal keratinocytes
- B Variable cleavage planes, intraepidermal, junctional and dermal in different lesions ✓
- C Cleavage exclusively through the lamina lucida
- D Subcorneal split with acantholytic cells
Correct answer: B. Variable cleavage planes, intraepidermal, junctional and dermal in different lesions
Explanation
Kindler syndrome, caused by FERMT1 (kindlin-1) mutations, is unique among inherited blistering disorders because the cleavage plane varies between lesions: intraepidermal, junctional and dermal splits may coexist. Blistering is trauma-induced, acral and childhood-limited, followed by photosensitivity and poikiloderma. Fixed single-plane splitting defines simplex (basal keratinocytes), junctional (lamina lucida) and dystrophic (below lamina densa) EB, and subcorneal acantholysis suggests pemphigus foliaceus.
Reference: Fitzpatrick's Dermatology, 9th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.