Of every possible solution for elastosis perforans serpiginosa, fractional carbon dioxide laser may be the best
Nov. 13, 2017
My initial attraction to dermatology, which still offers great appeal, is the challenge and joy of differential diagnosis and clinical-pathologic correlation. I learned quickly, however, that patients don’t want to be interesting — they want a specific diagnosis for one reason only – to get the right treatment, preferably curative.
Elastosis Perforans Serpiginosa (EPS) is a rare, distinctive perforating disease characterized by asymptomatic or pruritic keratotic papules configured in an arciform or serpiginous pattern. (1) Lesions are usually on the head, neck, and other flexural sites. Although lesions may resolve spontaneously, often with scars, they are usually long lasting. The clinical differential diagnosis includes calcinosis cutis, granuloma annulare, tinea corporis, annular sarcoidosis, and porokeratosis of Mibelli; these disorders are easily distinguished by pathologic examination. (2) Histologically, transepidermal elimination of elastic fibers is observed; in cases of penicillamine-induced EPS, calcification can be observed between the collagen fibers. (3)
EPS may be considered as either idiopathic, reactive (associated with disorders of connective tissue), or drug-induced (penicillamine). The associated disorders include Down syndrome, osteogenesis imperfecta, scleroderma, acrogeria, Ehlers-Danlos syndrome type IV (vascular type), Marfan syndrome, Rothmund-Thomson syndrome, cutis laxa, and pseudoxanthoma elasticum. (2) The association between EPS and Down syndrome is unknown; however, factors present in this syndrome such as premature skin aging, joint hyperelasticity and acrocyanosis may suggest an underlying connective tissue disorder. (4) In cases of EPS associated with penicillamine therapy, the characteristic skin lesions typically develop within the first year of treatment. (2) Penicillamine is a copper chelator that impairs the activity of lysyl-oxidase. Lysyl oxidase is an enzyme is required for dermal elastic fiber cross-linking; the resultant abnormal elastic tissue becomes extruded by transepidermal elimination. (5) Listed in OMIM (130100), familial forms of EPS have been reported with a probable autosomal dominant inheritance.
There are no effective standard treatments for EPS, and so far there have been only single reports of the use of different drugs for systemic and topical therapy . Topical treatments have included retinoids (tretinoin, tazartotene) and imiquimod. Oral isotretinoin, intralesional steroids, and narrow band UVB treatments have been utilized, but with variable results. Destructive treatment with cryosurgery and electrosurgery have been attempted. There are conflicting reports about the positive therapeutic effect of laser therapy, including CO2 laser, Er: YAG laser and pulsed dye lasers. Some researchers have demonstrated the effectiveness lasers, with remission after aggressive sessions with CO2 laser, while other studies failed to demonstrate a therapeutic response with these lasers, which have also caused scarring. (3) Photodynamic therapy (PDT) with 5-aminolevulinic acid (ALA) in a penicillamine-induced refractory EPS patient demonstrated marked improvement, but not complete clearing, as several papules persisted.
Kelati et al reported the use of a fractional carbon dioxide laser in a 40+-year-old woman with Wilson disease with penicillamine-induced EPS. The lesions demonstrated complete regression, without scarring, after 3 sessions. The authors contend that fractional ablative lasers can reach the deeper dermis while sparing enough surface of untreated skin for the healing process and prevent scarring. (7) I kept staring at the before and after images with disbelief (and joy) at the results.
I don’t know when I will see my next patient with EPS. But with Every Possible Solution to choose from, the fractionated carbon dioxide laser will be recommended posthaste.
1. de Rezende LN, et al. Elastosis perforans serpiginosa. Indian Dermatol Online J 2014; 5: 236-7.
2. Lee SH. Elastosis perforans serpiginosa. Ann Dermatol 2014; 26: 103-6.
3. Polańska A, et al. Elastosis perforans serpiginosa: A review of the literature and our own experience. Postepy Dermatol Alergo 2016; 33: 392-5.
4. Pereira AC, et al. Elastosis perforans serpiginosa in a patient with Down’s syndrome. As Bras Dermatol 2010; 85: 691-4.
5. Menzies S, Kirby B. Drug-induced elastosis perforans serpiginosa. BMJ Case Rep 2015; Dec 7;2015.
6. Wang D, et al. Effective treatment of d-penicillamine induced elastosis perforans serpiginosa with ALA-PDT. Photodiagnosis Photodyn Ther 2015; 12: 140-2
7. Kelati A, et al. Treatment of elastosis perforans serpiginosa using a fractional carbon dioxide laser. JAMA Dermatol 2017; 153; 1063-4.
All content found on Dermatology World Insights and Inquiries, including: text, images, video, audio, or other formats, were created for informational purposes only. The content represents the opinions of the authors and should not be interpreted as the official AAD position on any topic addressed. It is not intended to be a substitute for professional medical advice, diagnosis, or treatment.