Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO2 laser: A case and brief review
Jillian W Wong1, Erika M Summers2, Mark B Taylor3, Ronald M Harris4
Dermatology Online Journal 17 (9): 10

1. University of Utah School of Medicine
2. University of Utah Department of Dermatology
3. Gateway Aesthetic Institute and Laser Center, Salt Lake City, Utah
4. School of Medicine, University of California, Irvine, California


Abstract

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.



Introduction

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a condition first reported by Marsden et al in 1979 as a “comedo nevus of the palm” [1]. PEODDN was later given its name by Abell and Read in 1980, when they described a linear epidermal nevus localized on the inner foot [2]. 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]. PEODDN lesions are asymptomatic or mildly pruritic and consist of multiple filiform or verrucous, keratotic, brown to flesh colored papules, which can coalesce into linear plaques. The lesions are most often located on acral surfaces, with predominance over the flexural aspects of the hands and feet, palms and soles. Lesions have also been described on the trunk, following Blaschko lines [3, 5]. Although treatment of PEODDN is often unsuccessful, herein we report a case of PEODDN partially treated with a combination erbium/CO2 laser.


Case report

A 12-year-old healthy girl presented with 2 years of asymptomatic lesions, appearing first on her right hand. She subsequently developed similar lesions on her left hand, left plantar foot, bilateral axillae, and left upper cutaneous lip. Her mother expressed concern about the cosmetic effects of the lesions and sought treatment options.

On physical examination, yellow-brown verrucous papules coalescing into a plaque were noted on the patient’s left upper cutaneous lip. She also had similar verrucous papules in her bilateral axillae. On both her right hand and left foot, yellow to dirty brown, verrucous and filiform papules were noted to coalesce into linear plaques.


Figure 1 Figure 2
Figure 1. Histopathology of right axillary lesion reveals papular epidermal architecture with dilated eccrine sweat duct ostia and cornoid lamellae.

Figure 2. Histopathology of right axillary lesion reveals cornoid lamellae associated with dilated eccrine ducts.

Histopathology of a punch biopsy of a skin lesion under the patient’s right axilla demonstrated a mildly acanthotic and papillomatous epidermis. A cornoid lamellae overlying a dilated eccrine duct ostia, characteristic of PEODDN, was visualized (Figures 1 and 2).

Both the patient’s right hand and bilateral axillae were treated once with a combination erbium/CO2. The Derma K laser was used in CO2 erbium mode with a 0.2 mm spot size, 2940 nm and 10,600 nm wavelengths of laser energy, in focused mode, with a pulse duration of .25 ms, 12 hrz, 0.30 joules/cm2, a duty cycle of 5 percent, and 6 watts of CO2 laser energy on the axillae. Erbium mode on the right hand at 0.2 mm spot size, 0.50 joules/cm² and 12 hrz was also performed.


Figure 3 Figure 4
Figure 3. Right axilla. The image on the left demonstrates gradual resolution of axillary PEODDN lesions approximately 7 months after combined erbium/CO2 treatment. The image on the right demonstrates the initial PEODDN lesions under the right axilla.

Figure 4. Right hand. The image on the left demonstrates the initial PEODDN lesions between the first and second digits. The image on the right demonstrates partial resolution of PEODDN lesions 7 months after combined erbium/CO2 therapy.

The patient reported that the laser treatment was painful immediately after the procedure. However, 7 months after this therapy, she reported that the lesions had begun to disappear, particularly in her right axilla (Figure 3). She has not had any subsequent erbium/CO2 therapy or other alternative treatments. After 1 year, several verrucous papules have reappeared between the digits of her right hand (Figure 4).


Discussion

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 [3, 6]. PEODDN is also associated with many conditions including scoliosis, anhidrosis, seizure disorder, alopecia, left hemiparesis, developmental delay, and onychodysplasia [7]. Our patient is clinically healthy, without any of the aforementioned associated disorders.

A variety of therapies have been utilized to eradicate the papules and plaques of PEODDN. Without application of medications, some PEODDN lesions may spontaneously flatten over time [8]. Attempted treatments have included topical corticosteroids, tar, PUVA, UVB, and anthralin but have demonstrated little success [9]. Topical ointment, containing 10 percent urea, and sun exposure have provided mild improvement in the lesions [6]. Keratolytics and retinoids have been attempted as treatments because of the keratotic nature of the disorder. Treatment of the lesions with topical 0.025 percent tretinoin cream was shown to be ineffective in one case, but emollients appeared to be effective in softening the palmar skin lesions [4]. To our knowledge, systemic retinoids have not been attempted. Surgical excision of a focal and circumscribed lesion may be an option, but surgery is not a realistic modality for extensive lesions. Ultrapulsed CO2 lasers have been used effectively to cosmetically improve PEODDN lesions, particularly in more extensive cases not amenable to surgical excision [10].

We report the use of a combination erbium/CO2 laser as a potentially effective method of therapy for PEODDN. CO2 lasers providing super and ultra pulses were first used in the 1990s for facial resurfacing by providing high energy microbursts of ablation and minimal thermal damage to the skin [11]. Erbium: yittrium-alumninum garnet (YAG) lasers were later found to be 20 times more efficient than CO2 laser energy and have demonstrated efficacy by producing minor thermal damage to the dermis to stimulate growth of a new lamellar collagen layer [11]. Combination erbium/CO2 laser therapy is a relatively new combined therapy that has been used for epithelial resurfacing, wrinkle reduction, facial scars, sun damaged skin, and expedited healing for cosmetic surgery [11]. The innovative use of combined erbium/CO2 laser therapy may offer significant cosmetic improvement for future PEODDN patients. In our case, the patient had partial resolution of PEODDN lesions. However, we suggest that more aggressive therapy with multiple erbium/CO2 laser treatments may provide long-term success for PEODDN patients.

References

1. Marsden R, Fleming K, Dawber R. Comedo naevus of the palm — a sweat duct naevus? Br J Dermatol 1979; 101: 717-22. [PubMed]

2. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol 1980; 103: 435-441. [PubMed]

3. Warren RB, Verbov JL, Kokai GK. Porokeratotic eccrine ostial and dermal duct nevus. Pediatric Dermatology 2006; 23: 465-466. [PubMed]

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: 924-927. [PubMed]

5. Goddard DS, Rogers M, Frieden IJ, Krol AL, White CR, Jayaraman AG, Robinson-Bostom L, Bruckner AL, Ruben BS. Widespread porokeratotic adnexal ostial nevus: Clinical features and proposal of a new name unifying porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus. J Am Acad Dermatol. 2009; 61: 1060-69. [PubMed]

6. Cambiaghi S, Gianotti R, Caputo R. Widespread porokeratotic eccrine ostial and dermal duct nevus along Blaschko lines. Pediatric Dermatology. 2007; 24:162-167. [PubMed]

7. 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: 364-367. [PubMed]

8. Mazuecos J, OrtegaM, Rios JJ, Camacho F. Long-term involution of unilateral porokeratotic eccrine ostial and dermal duct naevus. Acta Derm Venereol 2003; 83:147-149. [PubMed]

9. Jamora MJ, Celis MA. Generalized porokeratotic eccrine ostial and dermal duct nevus associated with deafness. J Am Acad Dermatol. 2008; 59 (2 Suppl 1): S43-45. [PubMed]

10. Leung CS, Tang WM, Lam WY, Fung Wk, Lo K. Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: literature review and report on the efficacy of laser treatment. British Journal of Dermatology. 1998; 138: 684-688. [PubMed]

11. Chen WP. Oculoplastic Surgery: The Essentials. (Chapter 14: Laser Resurfacing: Dual Mode by Cary E. Feibleman). Thieme NY, NY (2001).

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Title:

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

Journal Issue:

Dermatology Online Journal, 17(9)

Author:

Wong, Jillian W;
Summers, Erika M;
Taylor, Mark B;
Harris, Ronald M

Publication Date:

2011

Publication Info:

Dermatology Online Journal, UC Davis

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