Porokeratotic eccrine ostial and dermal duct nevus

We report 2 cases of congenital porokeratotic eccrine nevus in a 5-year-old girl and a 7-year-old boy. The clinical manifestations were those of nevus comedonicus palmaris. The histologic picture consisted of comedo-like dilatations and cornoid lamellae involving the eccrine ostia and ducts. One of the cases showed anomalies in the dermal eccrine ducts. We believe that these lesions are eccrine hamartomas unrelated to porokeratosis. We review similar cases from the literature and discuss the differential diagnosis.     

Porokeratotic eccrine ostial and dermal duct naevus: report of 10 cases

Background Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a congenital hamartoma that has rarely been documented. Objectives To describe the clinical and histological features of 10 patients with PEODDN. Methods A retrospective study of 10 patients with PEODDN attended in our paediatric hospital between 1999 and 2009 was performed. The clinical and histopathological features were reviewed. Results Nine children and one adult were included in the study. In 6 cases the lesion was present at birth and they were unique in 5 cases and systematized in the other 5 cases. Two cases in the same family are reported. The lesions were most commonly found on the extremities, although the palms or soles were involved in 9 cases and only in one case there was not affection of this area. The lesions were mostly asymptomatic but there was moderate itching in two cases. Clinical examination revealed no extracutaneous involvement. Conclusions PEODDN is a rare disorder that usually appears at birth or early childhood. It has a linear distribution following the Blaschko lines. To our knowledge this is the largest series reported in English literature and we also describe the first family case.     

Porokeratotic eccrine ostial and dermal duct nevus

A 6-year-old girl with congenital porokeratotic eccrine ostial and dermal duct nevus involving the palmoplantar surfaces (ie, the back of the hands and feet), the limbs, the neck, and the axillae is described. The clinical and histopathologic findings are presented. This entity should be taken into consideration in the differential diagnosis of linear keratotic cutaneous eruptions.     

Porokeratotic eccrine ostial and dermal duct nevus

A case of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) affecting all 4 extremities is described. Clinically, palmo-plantar lesions resembled those of nevus comedonicus and the remaining lesions those of linear verrucous epidermal nevus. Histologically, all lesions were identical with cornoid lamellae in the eccrine ostia and epidermal ducts.     

Porokeratotic eccrine ostial and dermal duct nevus

Porokeratotic eccrine ostial and dermal duct nevus is a rare disorder characterized by distinctive histopathology. We describe a 6-year-old boy who had the typical palmar involvement and small discrete areas involving the midline of his back.     

Porokeratotic eccrine ostial and dermal duct nevus

Comedo-like lesions of the palm, present since birth, are studied electronmicroscopically and their eccrine nature demonstrated. We suggest their inclusion under the name porokeratotic eccrine-ostial and dermal duct nevus previously proposed by Abell and Read.     

汗孔角化性小汗腺孔和真皮导管痣

报告1例汗孔角化性小汗腺孔和真皮导管痣。患儿男,7岁。因手足和左膝关节内侧角化性丘疹和黑头粉刺样丘疹7年就诊。部分丘疹呈线状或带状排列（主要位于手足侧缘）。组织病理检查可见多个黑头粉刺样的表皮凹陷,内有角化不全柱。有些角化不全柱内可见扭曲的末端小汗腺导管穿过。角化不全柱的底部颗粒层变薄或消失,角质形成细胞空泡化,并可见角化不良细胞。角化不全柱下方的真皮内可见小汗腺导管。     

Porokeratotic eccrine ostial and dermal duct nevus

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.     

汗孔角化性小汗腺孔和真皮导管痣一例

患儿,男,13岁。因左上肢角化性丘疹及斑块8年,于2014年12月8日就诊于我科。8年前,在无明显诱因下左手掌尺侧缘小鱼际肌的表面出现少许角化性丘疹,无自觉症状。随后丘疹逐渐增多,部分丘疹融合成斑块,且呈带性分布。家族中未见类似疾病患者。     

Porokeratotic eccrine ostial and dermal duct nevus: a case of systematized involvement

We describe a case of porokeratotic eccrine ostial and dermal duct nevus presenting as widespread, symmetrical lesions. To our knowledge, this represents the most extensive case of this entity reported to date. Both the localized and systematized variants of this nevoid condition are discussed.     

Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: literature review and report on the efficacy of laser treatment

Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare, benign hamartomatous malformation involving the eccrine sweat duct. The existence of filiform keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts is a distinctive feature and the presence of associated abnormal dermal ducts is frequent. We report a patient with PEODDN who exhibited lesions on the left side of her chest. Cases of PEODDN reported in the literature are reviewed. Our experience in treating this patient with ultrapulsed carbon dioxide laser is also presented.     

A case of porokeratotic eccrine ostial and dermal duct naevus of late onset

We describe the case of a 21-year-old man with an 8-year history of porokeratotic eccrine ostial and dermal duct naevus (PEODDN) of late onset. The patient had pruritic, keratotic papules on the dorsal surface of his left hand, bilaterally on his palms and multiple yellowish lesions on the plantar surface of his feet. On histopathological examination cornoid lamella-like parakeratotic columns above eccrine sweat ducts were observed. The acrosyringium was also dilated. The lesions were refractory to treatment with salicylic acid in petrolatum and acitretin for 2 months. This entity should be taken into consideration in the differential diagnosis of linear keratotic cutaneous eruptions.     

Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO2 laser: a case and brief review

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon disease that presents early in childhood and is characterized by keratotic papules, often in a linear configuration. We describe a 12-year-old girl with characteristic lesions of PEODDN and describe her response to treatment with a combination CO2/Erbium laser. We also briefly review the literature on PEODDN.