Sakpuwadol Nawara, Suchonwanit Poonkiat
Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Clin Cosmet Investig Dermatol. 2025 Jan 6;18:31-36. doi: 10.2147/CCID.S500031. eCollection 2025.
Papular acantholytic dyskeratosis (PAD) of the vulva is an uncommon benign condition characterized by multiple hyperkeratotic papules in the anogenital region. First described in 1984, PAD belongs to the spectrum of focal acantholytic dyskeratoses and shares histopathological features with Darier disease and Hailey-Hailey disease. Despite its persistence, PAD is benign, requiring only reassurance in many cases. However, various treatment modalities have been reported for symptomatic patients, including topical and systemic therapies, and procedural interventions. We present a case of a 21-year-old Thai woman with asymptomatic perivulvar papules with typical histopathological features of PAD. After conservative management, the patient remained asymptomatic during follow-up. Additionally, we present a review of the current literature on this uncommon entity. This case highlights the importance of clinicopathological correlation in diagnosing PAD and distinguishing it from other clinically similar disorders. We discuss the clinical presentation, histopathological features, differential diagnosis, potential genetic associations, and management options for PAD.
外阴丘疹性棘层松解性角化不良（PAD）是一种罕见的良性疾病，其特征为在肛门生殖器区域出现多个角化过度丘疹。PAD于1984年首次被描述，属于局限性棘层松解性角化不良谱系，与达里埃病和黑利-黑利病具有共同的组织病理学特征。尽管PAD持续存在，但它是良性的，在许多情况下仅需给予安慰。然而，对于有症状的患者，已报道了多种治疗方式，包括局部和全身治疗以及手术干预。我们报告一例21岁泰国女性病例，其外阴周围有无症状丘疹，具有典型的PAD组织病理学特征。经过保守治疗，患者在随访期间仍无症状。此外，我们对关于这个罕见实体的当前文献进行了综述。该病例强调了临床病理相关性在诊断PAD以及将其与其他临床相似疾病区分开来方面的重要性。我们讨论了PAD的临床表现、组织病理学特征、鉴别诊断、潜在的基因关联以及治疗选择。
