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Distinguishing Histopathologic Features of Acantholytic Dermatoses and the Pattern of Acantholytic Hypergranulosis

Background: Numerous skin diseases exhibit the histopathologic finding of acantholysis. The extent and distribution of acantholysis has been regarded as a distinguishing feature of pemphigus vulgaris (PV) from other acantholytic conditions. Objective: To determine the extent and distribution of acantholysis in various acantholytic dermatoses and assess other potentially distinguishing histopathologic features. Methods: A cross-sectional retrospective review of the histopathologic features of PV, pemphigus foliaceus (PF), Hailey-Hailey disease (HHD), Darier’s disease (DD), Grover’s disease, and pityriasis rubra pilaris (PRP) was performed. Results: Biopsies of PV (n=49), HHD (n=27), DD (n=25), Grover’s disease (n=65), and PRP (n=33) showed suprabasilar acantholysis, limited to the lower epidermis in PV and PRP and involving all epidermal layers in HHD, DD, and Grover’s disease. Acantholysis in PF (n=38) mainly involved the upper epidermis. Follicular acantholysis occurred frequently in PV and PF compared with Grover's disease, HHD, and DD. Eccrine acantholysis was found in PV (42%), HHD (18%), PF (13%), and DD (4%). Grover's, DD, and HHD had greater dyskeratosis than PF, PV, and PRP. Neutrophils were more common in PV, PF, and HHD, while eosinophils were more common in Grover’s and DD. The pattern of acantholytic hypergranulosis occurred predominantly in PF. Conclusions: Adnexal acantholysis is not a distinguishing feature of pemphigus vulgaris. The level of acantholysis, degree of dyskeratosis, and acantholytic hypergranulosis are distinguishing features between the two types of pemphigus and other acantholytic disorders.

acantholysis ; pemphigus vulgaris ; pemphigus foliaceus ; Darier's disease ; Grover's disease ; Hailey-Hailey disease

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