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Pityriasis Rubra Pilaris With Extensive Follicular Acantholysis Resembling Pemphigus Vulgaris

A Case Report

Lilo, Mohammed T., MD*; Yan, Shaofeng, MD, PhD*; Chapman, Michael Shane, MD; Linos, Konstantinos, MD*

The American Journal of Dermatopathology: January 2019 - Volume 41 - Issue 1 - p 37–39
doi: 10.1097/DAD.0000000000001222
Brief Report

Abstract: Pityriasis rubra pilaris (PRP) is a rare, chronic, heterogeneous, papulosquamous inflammatory dermatosis of unknown etiology. Although erythematous scaly patches characterize the classic presentation of PRP, a broad range of clinical presentations has been reported. Histologically, PRP is characterized by psoriasiform acanthosis with alternating orthokeratosis and parakeratosis and rarely small acantholytic foci. In this article, we report a patient who presented with diffuse erythroderma and extensive acantholysis mimicking pemphigus vulgaris histologically.

*Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center and Geisel School of Medicine, Lebanon, NH; and

Section of Dermatology, Department of Surgery, Dartmouth-Hitchcock Medical Center and Geisel School of Medicine, Lebanon, NH.

Correspondence: Konstantinos Linos, MD, Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756 (e-mail: Konstantinos.linos@hitchcock.org).

The authors declare no conflicts of interest.

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