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Epidermolysis Bullosa Simplex — SCE Dermatology MCQ

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HardBlistering DiseasesEpidermolysis Bullosa SimplexSCE Dermatology

A 54-year-old develops trauma-induced tense blisters, milia and scarring on the hands. Direct immunofluorescence shows a u-serrated basement-membrane pattern and indirect immunofluorescence on salt-split skin binds the dermal floor. What is the diagnosis?

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Correct answer: A — Epidermolysis bullosa acquisita

A u-serrated pattern reflects autoantibodies beneath the lamina densa, most often against type VII collagen, and is characteristic of epidermolysis bullosa acquisita. Floor binding alone is not entirely specific because anti-p200 pemphigoid can also bind the dermal side; the serration pattern and mechanobullous scarring phenotype provide the discrimination.

Reference: https://academic.oup.com/bjd/pages/bad-guidelines