Indian Journal of Dermatopathology and Diagnostic Dermatology

: 2019  |  Volume : 6  |  Issue : 1  |  Page : 51--52

Generalized unilateral porokeratotic eccrine ostial and dermal duct nevus along the lines of blaschko: A rare entit

Abhishek Kumar Gupta1, Ankita Agrawal2, Kinjal Rambhiya2, Meena Makhecha2,  
1 Department of Dermatology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
2 Department of Dermatology, HBT Medical College and Dr. RN Cooper Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Ankita Agrawal
Department of Dermatology, HBT Medical College and Dr. RN Cooper Hospital, Mumbai, Maharashtra

How to cite this article:
Gupta AK, Agrawal A, Rambhiya K, Makhecha M. Generalized unilateral porokeratotic eccrine ostial and dermal duct nevus along the lines of blaschko: A rare entit.Indian J Dermatopathol Diagn Dermatol 2019;6:51-52

How to cite this URL:
Gupta AK, Agrawal A, Rambhiya K, Makhecha M. Generalized unilateral porokeratotic eccrine ostial and dermal duct nevus along the lines of blaschko: A rare entit. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2020 Jul 11 ];6:51-52
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Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a congenital hamartoma with eccrine differentiation. It is characterized by asymptomatic-grouped keratotic papules and plaques arranged in linear pattern usually localized on the palms and soles with distinct histopathological features. Here, we report a case of generalized PEODDN following the lines of Blaschko. A 22-year-old female presented with asymptomatic keratotic papules and plaques on the right side of the face, back, and right upper extremity including palm and nail since 4 years of age. There was no history of similar complaints in the family. Cutaneous examination revealed discrete keratotic papules, few papules coalescing to form hyperkeratotic plaques with central keratinous, comedo-like plugs, and pits in a linear configuration following the lines of Blaschko. These lesions were distributed linearly over the right palm extending above the wrist to the tips of fingers, forearm, arm, and right side of back not crossing the midline and right side of chin and cheek [Figure 1] and [Figure 2]. On dermoscopy, punctate pits filled with a keratin plug [Figure 3]a and involved nail showed yellow dot arranged in a linear fashion [Figure 3]b. Our clinical differential diagnosis was inflammatory linear verrucous epidermal nevus (VEN) and Blaschkoid PEODDN. General physical examination was normal. We took biopsy from papule over the palm and forearm and both revealed similar histopathology. Histopathological examination revealed orthokeratosis, and parakeratotic column filling a deep cup-shaped invagination in the epidermis [Figure 4]. Epidermis at the base of column showed loss of granular cells replaced by cluster of dyskeratotic cells. An eccrine duct was seen at the base of invagination consistent with acrosyringeal origin. The patient was given topical keratolytics in the form of 10% urea and 6% salicylic ointment.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

The PEODDN is a rare disorder of keratinization. The term “PEODDN” was introduced after the description of Abell and Read who reported PEODDN in 1980.[1] The disease is characterized histologically by well-formed cornoid lamellae occurring in association with dilated eccrine ducts and acrosyringia. It is composed of small keratotic papules or comedo-like pits filled with keratotic plugs in a linear distribution. PEODDN usually has unilateral distribution on the distal portion of a limb, on the palm or sole. A wider distribution of the disorder is a rare occurrence. Face and neck have not described in the literature but are found in our case. Association with other conditions is rare and includes neurological problems, scoliosis, palmoplantar keratoderma, onychodysplasia, alopecia, and hyperthyroidism. Evidence of the possible occurrence of the PEODDN along Blaschko lines as shown by our patient has been reported only in few cases.[2]

The lack of carcinoembryonic antigen expression by acrosyringial cells leads to development of the disease as proposed by Stoof et al.[3] Histopathology of PEODDN is diagnostic which shows epidermal invaginations containing cornoid lamella that involves the eccrine ducts. Differential diagnosis includes linear VEN, inflammatory linear VEN, linear porokeratosis, linear psoriasis, punctuate keratoderma, and nevus comedonicus. However, dissimilar to other forms of porokeratosis, this condition appears to be congenital and occurs exclusively in association with acrosyringia, the intraepidermal portions of theccrine sweat ducts. The course of PEODDN is usually stationary or sometimes progressive. Treatment of PEODDN is generally unsatisfactory.[4] Long-term involution of the lesions has been observed rarely. The lesions are benign and no malignant transformation had been reported. Small and localized lesions may be surgically excised. There was little success with topical corticosteroids, tar, psoralen ultraviolet A, ultraviolet B, anthralin, keratolytics, and retinoids. Good results with laser therapy, in the form of ultrapulse CO2 laser and combined erbium/CO2 laser, have been reported.[5] This case is reported due to rarity of generalized PEODDN.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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