AUTHOR=Le Stephanie T. , Toussi Atrin M. , Nava Jordan E. , Downing Lauren A. , Fung Maxwell A. 

TITLE=Case report: Mounded and refractory keratoses (MARK), a novel presentation of pemphigus vulgaris

JOURNAL=Frontiers in Medicine

VOLUME=9

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2022.1087382

DOI=10.3389/fmed.2022.1087382

ISSN=2296-858X

ABSTRACT=<p>Pemphigus vulgaris (PV) is a rare immunobullous disease. Although it classically presents as generalized flaccid blisters affecting the skin and mucosae, atypical cases of PV can be diagnostically challenging. Herein, we report an underrecognized non-blistering manifestation of pemphigus vulgaris, which we call mounded and refractory keratoses (MARK). MARK presents as exuberant scaling plaques on the scalp, often in the skin of color. When MARK features are present, pemphigus vulgaris is prone to misdiagnosis, clinically and histopathologically, leading to delays in appropriate treatment. Specifically, biopsies from these patients may resemble acantholytic dyskeratosis, resulting in initial misdiagnosis. Thus, recognizing this presentation may aid physicians in diagnosing and monitoring the recurrence of pemphigus vulgaris.</p>