Dupont Manon, Parent Muriel, Vanhooteghem Olivier
Dermatology Department, CHU UCL Namur, Site Sainte Elisabeth, Namur, 5000, Belgium.
Department of Pathology, Institute of Pathology and Genetics (IPG), Gosselies, 6041, Belgium.
Int J Womens Health. 2023 Mar 9;15:355-359. doi: 10.2147/IJWH.S398604. eCollection 2023.
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.
Dowling-Degos病（DDD）是一种罕见的遗传性皮肤病，表现为后天性褶皱部位的网状色素沉着。我们报告一例45岁女性病例，该患者自30岁起出现网状外观的色素沉着斑，位于外阴、肛周及双侧腋窝，同时伴有迟发性化脓性汗腺炎（HS），处于Hurley 2期。DDD典型表现于屈侧褶皱部位，据我们所知，文献中报道的外阴部位病例不足12例。我们推测DDD是易感患者发生HS的原因。事实上，这种关联似乎可以通过一种共同的病理生理机制来解释，该机制靶向Notch信号通路，参与表皮细胞的增殖和分化，可诱导HS的发生。DDD应被视为HS的一个合并因素。
