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Papular acantholytic dermatosis
Other Resources UpToDate PubMed

Papular acantholytic dermatosis

Contributors: Chelsea Kesty, Susan Burgin MD
Other Resources UpToDate PubMed


Papular acantholytic dermatosis (PAD), is a very rare, benign, and commonly asymptomatic dermatosis of the anogenital region. PAD presents with multiple discrete whitish, pink, or skin-colored papules that may coalesce to form plaques. They are usually asymptomatic, but pruritus or a burning sensation may be associated. Sites of predilection include the genital skin, perineum, perianal skin, and the inguinal folds. Lesions persist for months or years.

PAD is slightly more commonly reported in women than in men. It typically has onset in the third to seventh decade of life, although pediatric cases may rarely occur. PAD occurs sporadically.

The etiology and pathogenesis of PAD is still unknown. Some have suggested that PAD represents a localized form of Hailey-Hailey disease or Darier disease. Genetic mutations such as ATP2C1 and ATP2A2 have been reported in 3 cases, and in another case, PAD developed in a patient with a family history of Hailey-Hailey disease. Further investigation is needed to verify this hypothesis.


L11.8 – Other specified acantholytic disorders

726442001 – Acantholytic dyskeratosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Hailey-Hailey disease
  • Darier disease
  • Lichen planus
  • Candidiasis (Vulvovaginal candidiasis, Male genital candidiasis)
  • Genital wart
  • Seborrheic keratosis
  • Molluscum contagiosum
  • Pemphigus vegetans
  • Perianal pseudoverrucous papules and nodules
  • Bowenoid papulosis
  • Lichen simplex chronicus
  • Pearly penile papules

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Last Reviewed:02/26/2018
Last Updated:02/26/2018
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Papular acantholytic dermatosis
A medical illustration showing key findings of Papular acantholytic dermatosis : Erythema, Smooth plaques
Copyright © 2024 VisualDx®. All rights reserved.