Porokeratotic eccrine ostial and dermal duct nevus: A case report with brief review of literature.
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare benign cutaneous disorder. It has clinical resemblance with comedo nevus, usually presents in palms and soles. The disease is characterized histologically by well-formed cornoid lamellae occurring in close association with or overlying dilated eccrine ducts and acrosyringia. The lesions follow the Blaschko's lines and may be systematized. We describe a 20-year-old female with PEODDN.
Keywords: Porokeratotic eccrine ostial and dermal duct nevus, Blaschko's lines, linear.
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare benign cutaneous disorder. It has clinical resemblance with comedo nevus, usually present in palm and sole, where pilosebaceous follicles are absent. PEODDN was first reported by Marsden et al.1 in 1979 as a "comedo nevus of the palm". It was later given its name by Abell and Read in 1980,2 when they described a linear epidermal nevus localized on the inner foot. The disease is characterized histologically by well-formed cornoid lamellae occurring in close association with or overlying dilated eccrine ducts and acrosyringia.3 The lesions usually present at birth or in childhood, although cases of late-onset adult PEODDN have been described.3,4
A 20-year-old female presented with asymptomatic linear pits with comedo like plugs on left sole extending from heel along the medial border of foot upto tip of left great toe along the Blaschko's lines (Figure 1). Lesions were persistent from childhood, since 3 years of age and growing as the patient grows up. Her family history was unremarkable. Mucocutaneous examination shows normal oral mucosa, hair and nail. Her routine laboratory tests, serum chemistry panel and HIV serology were within normal limits. Histopathology showed epidermal acanthosis, deep epidermal invagination of a parakeratotic column (cornoid lamella), which is distinctive feature of PEODDN with absent granular layer . There was an eccrine unit in the dermis, in close approximation to the epidermal invagination (Figure 2).
The patient presented for follow-up after being placed on tretinoin 0.1% cream, 10% salicylic acid ointment and clobetasol propionate. After 2 months of treatment with these topical therapies, there was no observable improvement. The patient was then treated with radiofrequency cautery with notable improvement after two treatment sessions.
Porokeratotic eccrine ostial and dermal duct nevus is thought to be an eccrine hamartoma that presents at birth and persists into adulthood. But adult-onset POEDDN case have been described, and its frequency may be as high as 26%.5 Lesions of POEDDN are usually asymptomatic but mild pruritus may present. It predominantly occurs on the palms and soles as linear punctate pits or pitted papules. The condition is very rare and has a relatively equal occurrence in both genders. PEODDN clinically presents as either multiple linear punctuate pits with comedo-like plugs on palms and soles or keratotic plaques and papules that resemble linear VEN on other areas.6,7 But it may consist of multiple verrucous, keratotic, brown to flesh-colored papules often coalescing into linear plaques. The lesions follow the Blaschko's lines and may be systematized.8
The etiology of PEODDN is currently unknown, however, studies have alluded to a genetic contribution through genetic mosaicism and a possible eccrine or circumscribed epidermal keratinization abnormality.9,10 PEODDN is also associated with many conditions including scoliosis, anhidrosis, seizure disorder, sensory polyneuropathy, deafness, left hemiparesis, developmental delay, breast hypoplasia, alopecia, onychodysplasia, Bowen's disease, and squamous cell carcinoma.11,12 Our patient was clinically healthy, without any of the aforementioned associated disorders. The differential diagnoses include nevus comedonicus, punctate keratoderma, linear verrucous epidermal nevus, inflammatory linear verrucous epidermal nevus, and linear porokeratosis.12 PEODDN is usually differentiated from these entities by its characteristic presentation and histological presence of porokeratosis-like features in close association with the eccrine unit.
POEDDN is a benign condition, usually stationary or sometimes progressive but no malignant transformation has been reported. Topical management with topical corticosteroids, tar, psoralen ultraviolet A, ultraviolet B, anthralin, keratolytics, and retinoids may try but unsatisfactory. Surgical management with laser therapy, in the form of ultrapulse CO2 laser and combined erbium/CO2 laser have been reported.13 Lesions in our patient was deeply cauterized with radiofrequency cautery and the lesions subsided significantly.
1. Marsden R, Fleming K, Dawber R. Comedo naevus of the palm - a sweat duct naevus? Br J Dermatol. 1979;101:717-22.
2. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol. 1980;103:435-41.
3. Warren RB, Verbov JL, Kokai GK. Porokeratotic eccrine ostial and dermal duct nevus. Pediatr Dermatol. 2006;23:465-6.
4. Stoof TJ, Starink TM, Nieboer C. Porokeratotic eccrine ostial and dermal duct nevus. Report of a case of adult onset. J Am Acad Dermatol. 1989;20:924-7.
5. Valks R, Abajo P, Fraga J, Aragues M, Garcia-Diez A. Porokeratotic eccrine ostial and dermal duct nevus of late onset: More frequent than previously suggested? Dermatology. 1996;193:138-40.
6. Wang NS, Meola T, Orlow SJ. Porokeratotic eccrine ostial and dermal duct nevus: A report of 2 cases and review of literature. Am J Dermatopathol. 2009;31:582-6.
7. Leung CS, Tang WY, Lam WY, Lo KK. Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: Literature review and report on the efficacy of laser treatment. Br J Dermatol. 1998;138:684-8.
8. Masferrer E, Vicente MA, Bassas-Vila J, Rovira C, Gonzalez-Ensenat MA. Porokeratotic eccrine ostial and dermal duct naevus: Report of 10 cases. J Eur Acad Dermatol Venereol. 2010;24:847-51.
9. Warren RB, Verbov JL, Kokai GK. Porokeratotic eccrine ostial and dermal duct nevus. Pediatr Dermatol. 2006;23:465-6.
10. Cambiaghi S, Gianotti R, Caputo R. Widespread porokeratotic eccrine ostial and dermal duct nevus along Blaschko lines. Pediatr Dermatol. 2007;24:162-7.
11. Sassmannshausen J, Bogomilsky J, Chaffins M. Porokeratotic eccrine ostial and dermal duct nevus: A case report and review of the literature. J Am Acad Dermatol. 2000;43:364-7.
12. Pathak D, Kubba R, Kubba A. Porokeratotic eccrine ostial and dermal duct nevus. Indian J Dermatol Venereol Leprol. 2011;77:174-6.
13. Wong JW, Summers EM, Taylor MB, Harris RM. Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO2 laser: A case and brief review. Dermatol Online J. 2011;17:10.
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|Publication:||Journal of Pakistan Association of Dermatologists|
|Date:||Dec 31, 2017|
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