Acantholysis

What it means

The loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes.

Acantolysis results in a positive Nikolsky's sign in which slight rubbing of the skin results in exfoliation of the outermost layer.

Rare diseases that can present with this12

The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 1 disease where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.

Part of the broader category

Also called: Nikolsky's sign