Kumar Piyush, Mondal Ashim Kumar, Ghosh Kalyan, Mondal Avijit, Gharami Ramesh Chandra, Chowdhury Satyendra Nath
Medical College, Kolkata, India.
Dermatol Online J. 2012 Jan 15;18(1):10.
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare nonhereditary malformation of the eccrine duct. A relationship with linear porokeratosis is not yet established; some consider it as a rare variant of porokeratosis involving the acrosyringium, whereas others consider it a separate entity based on distinctive clinical features and histologic accentuation within ostial structures. A 7-year-old girl presented with multiple asymptomatic keratotic papules over her right palm, present since the age of 6 months. These papules were arranged in a linear distribution over the palm and middle finger of the right hand. Most of the papules were discrete. However, lesions on the middle finger coalesced to form a plaque. Histology revealed a keratin filled deep invagination of the epidermis, notable for a column of parakeratosis ("cornoid lamella"). The dermis was notable for dilated eccrine ducts and absent inflammation. Considering the clinical and histological evidence, a diagnosis of PEODDN was made. Its clinical resemblance to linear lichen planus and linear porokeratosis is discussed. Also, we provide a brief review of this rare condition.
汗孔角化性小汗腺孔和真皮导管痣(PEODDN)是一种罕见的小汗腺导管非遗传性畸形。与线状汗孔角化症的关系尚未确立;一些人认为它是累及顶泌汗腺导管的汗孔角化症的罕见变异型,而另一些人则基于独特的临床特征和孔内结构的组织学特征将其视为一个独立的实体。一名7岁女孩自6个月大起右手掌出现多个无症状角化性丘疹。这些丘疹在右手掌和中指呈线状分布。大多数丘疹是离散的。然而,中指上的皮损融合形成了斑块。组织学检查显示表皮有角质填充的深凹陷,以一列不全角化(“鸡眼样板”)为显著特征。真皮以扩张的小汗腺导管且无炎症为显著特征。综合临床和组织学证据,诊断为PEODDN。讨论了其与线状扁平苔藓和线状汗孔角化症的临床相似性。此外,我们对这种罕见疾病进行了简要综述。