Atypical Clinical Presentations of Blistering Disease, with Donna Culton, MD, PhD

For many providers in the field of dermatology, atypical presentations of blistering diseases such as bullous pemphigoid represent a challenge in terms of correct diagnosis.

Donna Culton, MD, PhD, professor of dermatology at the University of North Carolina at Chapel Hill, discussed the contents of her Maui Derm NP+PA Fall presentation ‘Blistering Diseases: What’s the Diagnosis’ regarding a series of challenging cases for dermatologists, nurse practitioners, and physician assistants.

“The first case is actually a case of an atypical form of pemphigus called pemphigus herpetiformis,” Culton said. “It's typically thought of as a subtype of pemphigus foliaceus, so skin-only but it's in a child. So we’re really just encouraging everyone to keep their minds open, because pemphigus is typically a disease of the middle aged patient population, but we can see it in children and especially in teens.”

Culton noted that in her experience, a lot of patients with pemphigus and particularly pemphigus foliaceus are in their late teen years. She urged attendees to keep this in mind given the atypical nature of certain clinical presentations of pemphigus.

“We also have a case of pemphigus that was recalcitrant to rituximab,” Culton said. “Many, many cycles of rituxumab. This is an adult patient and there are challenges in terms of when a patient fails rituximab and ways to manage that, when there are persistent lesions or potentially relapses.”

After discussing a few other examples of challenges to diagnosing blistering diseases, Culton was asked about the major takeaways from her talk at the conference that she hopes attendees will be more aware of afterward.

“I think with the atypical clinical presentations, the real takeaway is going to be that a biopsy for routine histology will not always help you, and you need to do the biopsy for direct immunofluorescence,” Culton explained. “...Of course, always take the patient history and do the exam, but if it comes to mind that it could be one of these non-blistering or atypical presentations, really think about the direct immunofluorescence.

She also urged attendees to consider drug induced-forms of disease and how to manage such conditions. Culton added that she is encouraged by recent data on omalizumab and dupilumab, 2 medications being studied for blistering disorders.

View the full interview segment posted above to learn more about these topics.

The quotes used in this summary were edited for the purposes of clarity. Culton reported being a principal investigator for Lilly and receiving royalties from UpToDate.