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Tazarotene Is an Effective Therapy for Elastosis Perforans Serpiginosa
J. David Outland, MD;
Timothy S. Brown, MD;
Jeffrey P. Callen, MD
From the University of Louisville, Louisville, Ky.
Arch Dermatol. 2002;138:169-171.
REPORT OF CASES
CASE 1
A 22-year-old woman presented with a 2-year history of an eruption on
the anterior aspect of her neck and right arm that was relatively asymptomatic.
She had a history of cystinuria, which had been treated with D-penicillamine
for several years, but the D-penicillamine therapy had been discontinued 2
years before the onset of the eruption. A biopsy was performed approximately
1 year before her presentation to our institution. The biopsy specimen demonstrated
clawlike downgrowths of epidermis surrounding collections of amorphous basophilic
debris and hyperplastic elastic fibers. Many elastic fibers were noted to
be pushing through epidermal channels (Figure
1), a finding that was consistent with the clinical diagnosis of
elastosis perforans serpiginosa (EPS). The patient was treated unsuccessfully
with several modalities in a sequential fashion, including liquid nitrogen
cryotherapy monthly for 6 months, topical tretinoin gel nightly for 2 months,
oral isotretinoin at dosages ranging from 40 to 60 mg/d for 15 weeks, and
2 sessions of carbon dioxide laser surgery. On physical examination, she was
noted to have erythematous annular and arcuate keratotic plaques on the anterior
aspect of her neck and right arm (Figure 2).
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Figure 1. Case 1. Biopsy specimen demonstrating
transepidermal elimination of altered elastic fibers.
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Figure 2. Patient 1 on presentation to our
institution with erythematous annular plaques on her neck.
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CASE 2
A 56-year-old woman was referred to our institution for evaluation of
a prutitic "nonhealing scar" that had been present on the posterolateral aspect
of the left side of her neck for 1 year. The lesions developed 1 month after
a revised facial rhytidectomy scar revision. The patient had been treated
with oral cephalexin for 2 weeks and topical erythromycin solution for several
weeks, without improvement. Her medical history revealed that she had been
treated with D-penicillamine for Wilson disease for more than 20 years. The
D-penicillamine therapy had been discontinued 2 months before her presentation
to our clinic.
Physical examination revealed multiple 2- to 7-mm crusted, erythematous,
ulcerated papules and plaques with an arcuate configuration. These lesions
were associated with a 6-cm-long scar on the posterolateral aspect of the
left side of the neck.
A biopsy specimen demonstrated a central focus of pseudoepitheliomatous
hyperplasia that appeared to be connected to the epidermis by a channel of
epithelium. Adjacent inflammation with dermal necrosis, both within and surrounding
the epithelia, was evident. An elastic stain demonstrated an increased concentration
of elastic fibers in the middermis extending to the epidermis. Many of the
elastic fibers appeared clumped and thickened.
The patient was treated unsuccessfully with cryotherapy approximately
6 times; high-potency topical corticosteroids, including clobetasol and halobetasol,
for 6 weeks; topical 0.1% tretinoin cream and topical 0.05% tretinoin solution
for about 2 months each; and several intralesional injections of triamcinolone
acetonide (4 mg/mL).
THERAPEUTIC CHALLENGE
Multiple therapies have been reported to be effective in the management
of EPS. However, none has been universally accepted as the treatment of choice.
Reported effective treatments include liquid nitrogen cryotherapy1 and oral isotretinoin therapy.2
Our patients were previously treated with many modalities, without success;
therefore, topical tazarotene therapy was initiated.
SOLUTION
Because multiple therapies had failed in both cases, the patients were
offered a trial of 0.1% tazarotene gel. Both patients agreed and began using
0.1% tazarotene gel at bedtime. At the 1-month follow-up visits, their disease
was somewhat improved. After 2 months of tazarotene therapy, the condition
of patient 1 was greatly improved (Figure
3) and that of patient 2 was moderately improved. After 4 more weeks,
patient 2 was almost free of active disease. Patient 2 then discontinued tazarotene
therapy, and her disease flared. Other topical retinoid preparations were
then tried, without improvement. Cryotherapy was tried a few more times, also
without improvement, and the patient was finally re-treated with tazarotene,
which flattened her lesions within 6 weeks. Patient 1 has tried to taper her
usage of tazarotene but notices flares on discontinuation.
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Figure 3. Patient 1 after 2 months of treatment
with 0.1% tazarotene gel.
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The only adverse effect observed in both cases was mild irritation.
However, the irritation subsided after a few weeks of therapy. Both patients
continue to use tazarotene daily for intermittent courses when their disease
flares.
COMMENT
Elastosis perforans serpiginosa is a disorder in which altered elastic
fibers are recognized as foreign material and are extruded through the epidermis
by transepidermal elimination. The result is a papular eruption that is usually
arranged serpiginously, annularly, or arcuately. Many conditions are associated
with EPS, including Down syndrome, Rothmund-Thomson syndrome, Ehler-Danlos
syndrome, Marfan syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum.
Also, patients treated with penicillamine are prone to develop EPS.3 Elastosis perforans serpiginosa usually occurs in
young adults and shows a predilection for the head and neck.
Tazarotene is the first receptor-selective topical retinoid approved
for the treatment of plaque psoriasis. It selectively targets the 
and  subtypes of retinoic acid receptors. Ninety percent of retinoid
receptors in the skin are of the subtype. Hofmann et al4
reported tazarotene's effectiveness in the treatment of congenital ichthyoses
in an open, intraindividually controlled, half-side investigation. Burkhart
and Burkhart5 reported tazarotene's effectiveness
in treating a patient with Darier disease who had responded poorly to other
agents. One mechanism of action of tazarotene in psoriasis is thought to be
attributable to the down-regulation of keratins 6, 10, and 16.4
Tazarotene also has a strong antiproliferative effect via the expression of
3 genes: tazarotene-induced genes 1 through 3.6
Tazarotene has a low systemic absorption and is rapidly metabolized and eliminated.7 The most common adverse effect reported is local irritation.
To our knowledge, this is the first report of EPS being successfully treated
with tazarotene. The mechanism of action of tazarotene in treating EPS is
unknown. Tazarotene may have comedolytic properties that allow for the unplugging
of transepidermal pores in this disease. Also, the blockage of retinoic acid
receptors may play a role in decreasing the proliferation in EPS.
AUTHOR INFORMATION
Accepted for publication July 19, 2001.
This study was presented as a poster at the annual meeting of the American
Academy of Dermatology, San Francisco, Calif, March 10-15, 2000.
Corresponding author and reprints: Jeffrey P. Callen, MD, Department
of Medicine, Division of Dermatology, University of Louisville, 310 E Broadway,
Suite 2A, Louisville, KY 40202-1745 (e-mail: jefca{at}aol.com).
REFERENCES
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1. Rosenblum GA. Liquid nitrogen cryotherapy in a case of elastosis perforans serpiginosa. J Am Acad Dermatol. 1983;8:718-721.
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2. Ratnavel RC, Norris PG. Penicillamine-induced elastosis perforans serpiginosa treated successfully
with isotretinoin. Dermatology. 1994;189:81.
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3. Mehregan AH. Elastosis perforans serpiginosa. Arch Dermatol. 1968;97:381-393.
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4. Hofmann B, Stege H, Ruzicka T, Lehmann P. Effect of topical tazarotene in the treatment of congenital ichthyoses. Br J Dermatol. 1999;141:642-646.
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5. Burkhart CG, Burkhart CN. Tazarotene gel for Darier's disease. J Am Acad Dermatol. 1998;38:1001-1002.
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6. Nagpal S, Patel S, Asano AT, Johnson AT, Duvic M, Chandraratna RAS. Tazarotene-induced gene 1 (TIG 1), a novel retinoic acid receptor–responsive
gene in skin. J Invest Dermatol. 1996;106:269-274.
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7. Foster RH, Brogden RN, Benfield P. Tazarotene. Drugs. 1998;55:705-711.
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SECTION EDITOR: GEORGE J. HRUZA, MD; ASSISTANT SECTION EDITORS: DEE
ANNA GLASER, MD; ELAINE SIEGFRIED, MD
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