ARTICLE
Since 1863 when Baerensprung [1] first described naevus unius lateralis,
various names and classifications have been attributed to this entity
because of the relatively wide range of clinical and histological presentations.
Verrucous epidermal nevi can be localized, inflammatory or systemic, associating
in this last case neurological, ocular, skeletal or other developmental
defects.
From a histological point of view, the linear verrucous epidermal nevus
(LVEN) is characterized by orthohyperkeratosis, acanthosis and papillomatosis,
and can be associated with an organoid nevus [2]. A particular form, called
inflammatory linear verrucous epidermal nevus (ILVEN), has been defined
as a pruritic, inflammatory, persistent lesion with histopathological
features resembling psoriasis [3]. Although the evolution of both entities
is benign (only one case of squamous cell carcinoma reported [4]), treatment
is required for cosmetic reasons. A relatively large number of therapeutic
choices are available: tretinoin and 5-fluorouracil [5, 6], acitretin
[7], calcipotriol [8-11], dithranol [3], podophyllin [12], CO2
laser [13], dye laser [14], dermabrasion, cryotherapy [12]. Difficulties
arise from the relatively unstable results because of a high recurrence
rate of the lesions.
Five patients with ILVEN and five patients with LVEN are presented with
their evolution after CO2 laser treatment.
Materials and methods
Clinical data
1. A 5-year old female patient had had an inflammatory verrucous epidermal
nevus (ILVEN) since the age of 18 months. Linear erythematosquamous lesions
in the inguinal fold, on the right thigh, as well as in the perigenital
and perianal region were present. She had already received treatments
such as calcipotriol and topical corticosteroids without significant results.
2. An 11-year old male patient had had an inflammatory verrucous linear
lesion of the left arm, forearm and hand since he was 5 years old. The
diagnosis of ILVEN was confirmed histologically.
3. A 14-year old female patient presented with linear hyperkeratotic papular
eruptions of the left arm, shoulder and inguinal fold, which were diagnosed
as LVEN.
4. A 30-year old male patient had an LVEN of the right upper and lower
limbs and trunk. The lesions were congenital. Treatment with topical corticoids
and keratolytics was not effective.
5. A 34-year old male patient had an ILVEN of the left inguinal fold,
scrotum, upper thigh and popliteal fold. These lesions appeared at the
age of 7 and presented serious discomfort to the patient, due to the pruritus
and burning sensations. Acitretine treatment did not improve the symptoms.
6. The sixth patient was a 41-year old male who had had pruritic linear
erythematosquamous lesions on his left leg since the age of 10. He was
treated with topical corticosteroids without acceptable cosmetic results.
He complained of great discomfort because of pruritus, dryness, scaling,
and severe irritation.
7. A 6-year old female patient presented with an inflammatory linear verrucous
epidermal nevus of the right lower limb.
8. Since she was 3 years old, a 39-year old female of phototype IV had
a LVEN of the left side of the abdomen, which appeared as a linear lesion.
9. A 7-year old girl of phototype II had a LVEN on the left inferior eyelid,
of only few centimetres in size.
10. A 10-year old girl of phototype III had a LVEN of the right cheek.
In all our patients there was no clinical evidence of neurological,
ocular, skeletal defects or alteration of other organs. Clinical diagnosis
was confirmed by a histological examination for all patients with ILVEN.
Methods
Laser therapy
Management by superpulsed CO2 laser was performed as follows:
we started with a test treatment in order to appreciate the quality of
healing and, in the case of satisfactory results, we continued two months
later by removing the lesion in several sessions. The skin was prepared
with an antiseptic such as Hexamidine or Betadine®. Wet
towels were placed around the treatment field. The patient was given laser
safety glasses, and stainless steel eye shields were placed under the
eyelids if the eyelids were to be treated. The treatment was performed
with local anaesthesia with EMLA® cream. Two children (patients
no. 1 and 7) needed general anaesthesia and one week hospitalization.
A Sharplan (Sharplan-ESC Medical system Ltd, Yokneam, Israël) CO2
laser of 20 watts, with a hand piece of 200 mm, was used in the "Silk
Touch" modality (Sharplan Lasers, Inc., Allendale, New Jersey 07401,
USA).
The carbon dioxide lasers have been limited by the tendency of the laser
to leave behind a zone of coagulation necrosis around the treatment site,
which measured up to 1 mm across. This often results in unacceptable scarring.
The carbon dioxide laser emits light at 10,600 nm. This long infrared
wavelength is strongly absorbed by water, which is the main component
of skin. The laser light is absorbed within 20 µm of tissue. But
with the conventional carbon dioxide laser, the surrounding tissue is
also heated up through heat conduction away from the impact site. To confine
the damage to the impact site, the laser energy must be delivered within
a period of time short enough that the thermal conduction away from the
impact is minimal. The desirable zone of thermal damage is 50 to 100 µm.
This is small enough to successfully seal small dermal blood vessels and
to maintain a bloodless field. To confine the thermal energy, pulse duration
of approximately 1 msec is necessary, and enough energy must be delivered
to completely vaporize the water in the target tissue. The "Silk Touch"
laser delivery system is a microprocessor controlled miniature optomechanical
flashscanner compatible with most CO2 lasers. The "Silk Touch"
consists of two orthogonally vibrating mirrors and a focusing lens. Reflections
of the CO2 laser optical beam from the mirrors cause the beam
to deviate from its original direction by an angle: théta. The
microprocessor-controlled mirrors are programmed to spatially and non
linearly vary by théta, and generate a rapidly collapsing spiral
curve on tissue with a constant tangential linear velocity, which gives
a homogeneous vaporisation. Using this method, the beam is scanned in
a spiral pattern so that any given spot of tissue is irradiated for less
than 1 msec with an energy density of 5 to 15 J/cm2 or more.
The effect is as if the lasers were pulsed.
In all but patients no. 1 and 7, we chose a pulsed power of 15 watts
and an exposure time of 0.45 seconds. For each region of the nevi, two
passages were carried out during the same session. Between the two passes,
the resulting desiccated tissue debris was wiped away with saline soaked
dry gauze. Additional passes were carried out over the remaining lesions
to smoothen them, but with increasing risk of complications. In cases
1 and 7, the procedure was performed using only one passage of 14 watts
or less pulsed power, with 0.4 seconds exposure, because of the younger
age of these patients.
Post-treatment care and follow-up
During the healing process, daily applications of vaseline and antibiotic
ointments (sulfadiazin argentic FLAMMAZINE®, or fucidic
acid FUCIDINE®) on the treated zone were performed. Follow-up
was extended to a period of 2 years. Additional sessions of CO2
laser were performed in order to eradicate the entire nevus or to treat
recurrences.
Results
Patient no. 1 showed a complete recurrence of the erythematosquamous
plaques one month after the first therapy session. In this case, the procedure
was discontinued and our option was to prescribe local applications of
calcipotriol and betametasone.
Concerning patient no. 2, four sessions of CO2 laser were
necessary in order to remove the entire lesion. Healing was achieved with
a satisfactory cosmetic result (Fig. 1A, B). Only minor relapses
consisting of erythematous papules were visible at the end of our observation.
Four sessions were needed for the treatment of our third patient. The
healing process left a slight hyperpigmentation in this person with a
skin phototype V (Fig. 2A, B).
A very good evolution was shown by the fourth patient in whom only the
inguinal and axillary folds were treated.
In patient no. 5, scarring was aesthetic in the popliteal fold and on
the upper thigh with a complete regression of the nevus; a few erythematous
papules of 0.5 cm diameter appeared in the perigenital region for which
a minor intervention was necessary. In addition, the patient experienced
no further pruritus.
The sixth patient was treated only partially (Fig. 3) evolution
was cosmetically satisfactory with minor desquamation of the scar and
absence of pruritus.
In our seventh patient, the entire nevus was removed during one session,
using a single passage. After one year, the lesion completely recurred
with only a few areas of normal appearing skin.
The last two patients had entirely successfully results of the treatment,
with only one passage.
Discussion
Few data are available concerning the aetiology of LVEN. The skin lesions,
which are typically linear, are considered as clones of cells carrying
an unknown defect. Welch et al. [15] have demonstrated the similarity
of ILVEN to psoriasis from a histological and immunohistochemical point
of view, while Ginarte et al. [16] individualised unilateral psoriasis
by involucrin immunohistochemistry. Happle et al. [17] have recently
demonstrated that CHILD nevus, classified as an epidermal nevus syndrome,
is in fact an X dominant defect of cholesterol biosynthesis. Molecular
biology will presumably offer clues about the pathogenesis of LVEN and
ILVEN.
Numerous treatments have been suggested for epidermal nevi, each shown
to be unsatisfactory. The limit of medical approaches is the great rate
of relapses while surgical techniques leave permanent scars. The mechanism
of action of the various substances, which have been proposed to heal
epidermal nevi, is not entirely understood. Calcipotriol may exert a modulation
of the production and action of cytokines and of the promotion of the
epidermal differentiation in the same way as in psoriasis [11]. The chemotherapeutic
agent 5-fluorouracil has an antiproliferative effect by inhibition of
DNA synthesis [6]. Keratolytic agents, topical steroids, tars and tretinoin
may act on some pathogenetical pathways without resolving the complex
epidermal and dermal defects.
The aim of the different surgical techniques is to destroy the entire
lesion and if possible without cosmetic impairment. Surgical resection
is the most reliable treatment for lesions having a limited surface. Treatment
by 585 nm flashlamp-pumped dye laser improved the symptoms in one case
reported by Alster (1994) and the result was stable for over one year
follow-up [14]. Landthalter et al. (1984) successfully treated
two patients with localized verrucous nevi by argon laser [18]. Using
the same method, Hohenleutner and Landhaler [19] achieved very good to
acceptable results in 85% of their patients with papillomatous lesions,
but failed to heal 'hard' verrucous nevi. In some of these cases, CO2
laser was effective, whereas generally there was a tendency to hypertrophic
scar formation. The same authors [20] report the complete removal by CO2
laser, without scarring or recurrence of a widespread epidermal nevus.
Ratz et al. [21] have also used this technique, which proved to
be effective in 15 patients with epidermal nevi.
We present ten patients with epidermal nevi, type LVEN and ILVEN, treated
by CO2 laser. The interest of our technique is to repeat the
application at low power for limiting the scarring with the CO2
laser. We preferred to perform two passages at lower energy to try to
avoid scars. Both patients with verrucous epidermal nevi had a good evolution
without relapses; these relatively superficial lesions responded optimally
with complete regression, leaving hyperpigmented scars but no hypertrophy.
The more profound (mid dermis) inflammatory lesions of ILVEN needed
a more aggressive treatment. The underlying dermis must be removed or
destroyed thus enhancing the risk of suboptimal cosmetic results. In our
patients, pruritus disappeared while erythema and keratosis diminished
progressively after each session of CO2 laser treatment. Resulting
wounds healed without complication. A 2-year follow-up showed that 3 patients
with ILVEN experienced no significant recurrence in the treated areas.
In two children (cases no. 1 and 7), in which lesions recurred perigenitally,
perianally and on the lower limbs respectively, we used lower parameters
of the laser (pulse duration less than 0.45 sec and energy density under
or equal to 14 J/cm2). Also, we performed only one single passage
on each area. In these two cases, we decided not to continue the procedure
because of the scarring risk but also because anaesthesia might have been
needed in these two young children if a more aggressive technique was
chosen.
Article accepted on 3/5/01
CONCLUSION We
conclude that appropriate laser power and sufficient vaporization including
deep dermal inflammation are the clues for successful laser treatment of
ILVEN. This treatment of a benign tumorous lesion is justified because of
the functional consequences and of the lack of other solutions. In our experience,
carbon dioxide laser is an effective and safe treatment of verrucous epidermal
nevi provided that optimal technical conditions are fulfilled. Suboptimal
procedures lead to recurrences. This seems to be the only treatment to offer
a definitive reduction of ILVEN. In comparison with other laser therapies,
CO2 lasers have more favourable results but more scarring risk.
Concerning ILVEN, dye laser of the third generation could be the future
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