Acantholytic dyskeratosis of the vulva presenting with clinical features of vulval lichen sclerosus—A possible rare collision entity

We present a challenging case of chronic, erosive, scarring dermatosis of the vulva with clinical features of long standing lichen sclerosus (LS), namely pallor and loss of vulval architecture, but with histopathology consistently showing features of an acantholytic process. The history and clinical...

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Veröffentlicht in:Journal of cutaneous pathology 2020-01, Vol.47 (1), p.61-64
Hauptverfasser: Haddadeen, Ciara, Theaker, Jeffrey, Rowen, David, Lotery, Helen
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creator Haddadeen, Ciara
Theaker, Jeffrey
Rowen, David
Lotery, Helen
description We present a challenging case of chronic, erosive, scarring dermatosis of the vulva with clinical features of long standing lichen sclerosus (LS), namely pallor and loss of vulval architecture, but with histopathology consistently showing features of an acantholytic process. The history and clinical features of this case do not resemble other acantholytic conditions such as pemphigus vulgaris, Hailey‐Hailey disease, Darier disease, or the entities described as acantholytic dermatoses affecting the vulva. As far as we are aware, the combination of the clinical features and histopathologic findings in our case do not fit with any previously described condition and we propose that this is a rare entity of a collision of LS and an erosive acantholytic process occurring together.
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subjects Darier's disease
Dyskeratosis
focal acantholytic dyskeratosis
Keratosis
lichen sclerosus
papular acantholytic dyskeratosis
Pemphigus
Vulva
title Acantholytic dyskeratosis of the vulva presenting with clinical features of vulval lichen sclerosus—A possible rare collision entity
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