Hailey-Hailey Disease

The last 7 months have been pretty busy as I’ve been working on a new laboratory here in Knoxville and now I’m finally getting a bit of time where I can hopefully start posting regularly again. We received a really good case of Hailey-Hailey disease the other day that demonstrated the classic dilapidated brick wall appearance. This biopsy was from the axilla of a middle aged woman, which is a classic location. Still trying to get my camera settings right. I am using an Infinity2 Lumenara camera paired with an Olympus microscope and LED light source. I receive no royalties for endorsing this microscope, but I really like the LED light source, it’s easy on the eyes. Not sure if it reflects well in the photos, but I end up manipulating the brightness & contrast anyway so it probably doesn’t make a difference.

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5 responses to “Hailey-Hailey Disease

  1. There is quite a bit of inflammation in the dermis. In a genetic disorder of desmoplakin formation is that usually less often found than in autoimmune damage to these intercellular proteins? Excellent images Neil.

    • Great question. In most lesions of Hailey-Hailey disease I have seen, they are usually quite inflamed because they can be irritated either from erosion, blisters, contact or secondary infection. Quantity of inflammation may not help distinguish from pemphigus vulgaris, so I suggest doing immunofluorescence studies typically and correlation with the clinical findings. Some texts suggest presence or absence of eosinophils may help, but I’m sure they can be present in either case. Thanks Ian.

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