Dowling‐Degos disease with diffuse penile pigmentation

Taşkapan, Oktay; Gökçe, Elif; Akın, Osman; Erkilinc, A.C.; Demirkesen, Cüyan

doi:10.1111/jdv.12367

Cited by 3 publications

(1 citation statement)

References 9 publications

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“…2 This location, mainly reported among women, is also reported among men on the penis or scrotum. 3 DDD should be included in the differential diagnosis of vulvar hyperpigmentation with acanthosis nigricans and lentiginosis syndromes (Laugier's disease, Leopard's disease, Peutz Jeghers disease or Carney's complex). 4 DDD may be associated with HS.…”

Section: Discussionmentioning

confidence: 99%

About a Rare Association Between Vulvar Dowling Degos Disease and HS

Dupont¹,

Parent²,

Vanhooteghem³

2023

IJWH

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Dowling Degos disease (DDD) is a rare genodermatosis that manifests itself as acquired, reticulated hyperpigmentation of the folds. We report the case of a 45-year-old woman who presented since the age of 30 with hyperpigmented macules of reticulated appearance of vulvar, perianal and bilateral axillary location associated with hidradenitis suppurativa (HS) at Hurley stage 2 of later onset. DDD is classically described in the flexural folds and, to our knowledge; less than a dozen cases of vulvar location are published in the literature. We postulate that DDD is responsible for the development of HS in susceptible patients. Indeed, this association seems to be explained by a common pathophysiological mechanism, targeting the Notch signalling pathway, involved in the proliferation and differentiation of epidermal cells that can induce the development of HS. DDD should be considered as a comorbid factor of HS.

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Section: Discussionmentioning

confidence: 99%