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Hailey–Hailey–Like Pattern of Acantholysis on the Scalp Should Raise the Possibility of Incipient Pemphigus Vulgaris

Gottesman, Silvija P., MD*,†; Abedi, Syed M., MD; Rosen, Jason R., DO; Gottlieb, Geoffrey J., MD

The American Journal of Dermatopathology: April 2019 - Volume 41 - Issue 4 - p 286–288
doi: 10.1097/DAD.0000000000001298
Original Study

Abstract: Hailey–Hailey disease (familial benign chronic pemphigus) is a genodermatosis with a typical clinical presentation of macerated plaques involving intertriginous areas of the body and affects multiple family members. Epidermal acanthosis, incipient, and completed acantholysis of many of the spinous keratinocytes of the epidermis and foci of dyskeratosis are well-recognized histologic findings. We have observed solitary lesions on the “scalp” of patients having a Hailey–Hailey–like pattern, and not the suprabasilar clefting of pemphigus represents the first manifestation of pemphigus vulgaris, which was confirmed with direct immunofluorescence in all our patients.

*Zucker School of Medicine at Hofstra/Northwell, Lake Success, NY; and

Ackerman Academy of Dermatopathology, New York, NY.

Correspondence: Silvija P. Gottesman, MD, 1991 Marcus Avenue, Suite 300, Lake Success, NY 11042 (e-mail:

The authors declare no conflicts of interest.

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