Download (PUVA) for Dermatological Conditions

National Medical Policy
Subject:
Phototherapy and Photochemotherapy (PUVA)
For Dermatological Conditions
(Refer to the Health Net National Medical Policy Phototherapy for Psoriasis
for additional information)
Policy Number:
NMP441
Effective Date*: November 2008
Updated:
January 2015
This National Medical Policy is subject to the terms in the
IMPORTANT NOTICE
at the end of this document
For Medicaid Plans: Please refer to the appropriate Medicaid Manuals for,
coverage guidelines prior to applying Health Net Medical Policies
The Centers for Medicare & Medicaid Services (CMS)
For Medicare Advantage members please refer to the following for coverage
guidelines first:
Use
X
Source
National Coverage Determination
(NCD)
National Coverage Manual Citation
Local Coverage Determination (LCD)*
Article (Local)*
Other
None
Reference/Website Link
Skin Lesion Removal (Includes AK and Excludes
MOHS):
http://www.cms.gov/medicare-coveragedatabase/search/advanced-search.aspx
Use Health Net Policy
Instructions
 Medicare NCDs and National Coverage Manuals apply to ALL Medicare members
in ALL regions.
 Medicare LCDs and Articles apply to members in specific regions. To access your
specific region, select the link provided under “Reference/Website” and follow the
search instructions. Enter the topic and your specific state to find the coverage
determinations for your region. *Note: Health Net must follow local coverage
determinations (LCDs) of Medicare Administration Contractors (MACs) located outside their
service area when those MACs have exclusive coverage of an item or service. (CMS
Manual Chapter 4 Section 90.2)

If more than one source is checked, you need to access all sources as, on
occasion, an LCD or article contains additional coverage information than
contained in the NCD or National Coverage Manual.
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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
If there is no NCD, National Coverage Manual or region specific LCD/Article,
follow the Health Net Hierarchy of Medical Resources for guidance.
Current Policy Statement
Health Net, Inc. considers phototherapy with Ultraviolet A or B therapy or
photochemotherapy (PUVA) medically necessary when there has been a failure or
contraindication to treatment using conventional therapy for any of the following
medical conditions:

Severe atopic dermatitis (atopic eczema); or

Severe lichen planus; or

Severe photodermatoses (e.g., polymorphic light eruption, actinic prurigo,
chronic actinic dermatitis); or

Cutaneous T cell lymphoma (early stage mycosis fungoides); or

Severe large plaque parapsoriasis; or

Sclerotic Skin Diseases (e.g., Localized scleroderma, eosinophilic fasciitis,
chronic graft-versus-host disease, lichen sclerosus et atrophicus, scleredema
adultorum, necrobiosis lipoidica, POEMS disease, pansclerotic porphyria cutanea
tarda, and drug-induced scleroderma-like disorders); or
Health Net, Inc. considers Ultraviolet B (UVB) radiation (phototherapy) or topical or
oral psoralen ultraviolet A (photochemotherapy or PUVA) medically necessary for the
treatment of Vitiligo. Refer to the Health Net Medical Policy on Vitiligo Treatment
NMP32 for additional information.
Abbreviations
UV
UVA
UVB
PUVA
BB-UVB
NB-UVB
GVHD
IFN-y
SLEDAI
SLAM
PL
PLEVA
PLC
Ultraviolet Radiation
Ultraviolet Type A Radiation
Ultraviolet Type B Radiation
Combination of psoralen (P) and long-wave ultraviolet radiation (UVA)
Broad Band Ultraviolet Type B Radiation
Narrow Band Ultraviolet Type B Radiation
Graft versus Host Disease
Intracytoplasmic interferon y
SLE Disease Activity Index
SLE Activity Measure
Pityriasis Lichenoides
Pityriasis Lichenoides et Varioliformis Acuta
Pityriasis Lichenoides Chronica
Codes Related To This Policy
NOTE:
The codes listed in this policy are for reference purposes only. Listing of a code in
this policy does not imply that the service described by this code is a covered or noncovered health service. Coverage is determined by the benefit documents and
medical necessity criteria. This list of codes may not be all inclusive.
On October 1, 2015, the ICD-9 code sets used to report medical diagnoses and
inpatient procedures will be replaced by ICD-10 code sets. Health Net National
Medical Policies will now include the preliminary ICD-10 codes in preparation for this
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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transition. Please note that these may not be the final versions of the codes and
that will not be accepted for billing or payment purposes until the October 1, 2015
implementation date.
ICD-9 Codes
202.1
691
691.8
692
692.9
696.2
697.0
701
701.0
709.1
709.9
Mycosis fungoides (T-cell lymphoma)
Atopic dermatitis and related conditions
Other atopic dermatitis and related conditions [severe refractory]
Contact dermatitis and other eczema
Contact dermatitis and eczema of unspecified cause
Parapsoriasis
Lichen Planus
Other hypertrophic and atrophic conditions of skin
Circumscribed scleroderma
Vitiligo
Unspecified disorder of skin or subcutaneous tissue (dermatoses NOS)
ICD-10 Codes
C84.00C84.09
H02.731H02.739
L20.0L20.9
L23.0L23.9
L24.0L24.9
L25.0L25.9
L30.9
L41.0L41.9
L43.0L43.9
L80
L94.0
L98.9
Mycosis fungoides
Vitiligo of eyelid and periocular area
Atopic dermatitis
Allergic contact dermatitis
Irritant contact dermatitis
Unspecified contact dermatitis
Dermatitis, unspecified
Parapsoriasis
Lichens planus
Vitiligo
Localized scleroderma [morphea]
Disorder of the skin and subcutaneous tissue, unspecified
CPT Codes
96567
96910
96912
96913
Photodynamic therapy by external application of light to destroy
premalignant and/or malignant lesions of the skin and adjacent
mucosal (Eg. lip) by activation of photosensitive drug9(s), each
phototherapy session.
Photochemotherapy; tar and ultraviolet B (Goeckerman treatment) or
petrolatum and ultraviolet B
Photochemotherapy; psoralens and ultraviolet A (PUVA)
Photochemotherapy (Goeckerman and/or PUVA) for severe
photoresponsive dermatoses requiring at least four to eight hours of
care under direct supervision of the physician (includes application of
medication and dressings) (when specified as PUVA)
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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HCPCS Codes
A4633
E0691
E0692
E0693
E0694
Replacement bulb/lamp for ultraviolet light therapy system, each
Ultraviolet light therapy system panel, includes bulbs/lamps, timer, and
eye protection; treatment area two square feet or less
Ultraviolet light therapy system panel, includes bulbs/lamps, timer, and
eye protection, four foot panel
Ultraviolet light therapy system panel, includes bulbs/lamps, timer, and
eye protection, six foot panel
Ultraviolet multidirectional light therapy system in 6 foot cabinet,
includes bulbs/lamps, timer, and eye protection
Scientific Rationale – Update March 2011
Phototherapy is defined as exposure to nonionizing radiation for therapeutic benefit.
It can encompass the use of visible light, photodynamic therapy (PDT),
photothermolysis, and laser therapy. More specifically, visible light phototherapy
utilizes ultraviolet-free light within the visible spectrum, such as blue and red visible
light, with wavelengths spanning 415 to 660 nm. PDT is characterized by the use of
visible light in addition to a topical application of photosensitizers, such as commonly
used agent, 5-aminolevulinic acid (ALA) and its methyl ester (MAL). Another type of
phototherapy is photothermolysis, which uses both light and heat energy with
broadband intense pulsed light (IPL). Near-infrared lasers with 1320- to 1540-nm
wavelengths are also used. Other laser types used are pulsed dye laser (PDL), longPDL, argon laser, and diode laser. It has been suggested that solar, artificial
ultraviolet (UV), and psoralen plus ultraviolet A (PUVA) light should no longer be
employed for phototherapy acne treatment due their low efficacy and their potential
carcinogenic and photoaging effects.
According to the National Institutes of Health (NIH), National Institute of Arthritis
and Musculoskeletal and Skin Diseases (NIAMS) booklet Handout on Health: Atopic
Dermatitis (2009): “Atopic dermatitis is a chronic (long-lasting) disease that affects
the skin. It is not contagious; it cannot be passed from one person to another. The
word “dermatitis” means inflammation of the skin. “Atopic” refers to a group of
diseases in which there is often an inherited tendency to develop other allergic
conditions, such as asthma and hay fever. In atopic dermatitis, the skin becomes
extremely itchy. Scratching leads to redness, swelling, cracking, “weeping” clear
fluid, and finally, crusting and scaling. In most cases, there are exacerbations
followed by remissions. As some children with atopic dermatitis grow older, their skin
disease improves or disappears altogether, although their skin often remains dry and
easily irritated. In others, atopic dermatitis continues to be a significant problem in
adulthood. Atopic dermatitis is often referred to as “eczema,” which is a general term
for the several types of inflammation of the skin. Atopic dermatitis is the most
common of the many types of eczema. Treatment of atopic dermatitis includes the
use of ultraviolet A or B light waves, alone or combined, which can be an effective
treatment for mild to moderate dermatitis in older children (over 12 years old) and
adults. A combination of ultraviolet light therapy and a drug called psoralen can also
be used in cases that are resistant to ultraviolet light alone.
Tzaneva et al. (2010) completed a randomized observer-blinded crossover trial with
40 patients, to compare UVA1 and oral 5-methoxypsoralen (5-MOP) plus UVA with
respect to efficacy, tolerability and duration of response in patients with severe
generalized atopic dermatitis (AD). The patients received either 15 exposures to
medium-dose UVA1 as the first treatment and, in cases of relapse, another 15
exposures to 5-MOP plus UVA as the second treatment, or vice versa. All patients
were followed until 12 months after discontinuation of the last treatment. The
SCORAD score was determined by a blinded investigator at baseline, after 10 and 15
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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treatments each and during the follow-up period. In addition, all adverse events
were recorded during the whole study period. Twenty-three patients completed the
crossover treatment. Both phototherapies resulted in clinical improvement; however,
PUVA reduced the baseline SCORAD score to a significantly greater extent than UVA1
(mean +/- SD 543 +/- 257% vs. 377 +/- 228%; P = 0041). The median length of
remission was 4 weeks (interquartile range 4-12) after UVA1 and 12 weeks
(interquartile range 4-26) after PUVA therapy (P = 0012). PUVA provides a better
short- and long-term response than medium-dose UVA1 in patients with severe AD.
Scientific Rationale – Update January 2010
Vitiligo is an acquired skin depigmentation that produces white patches and can
affect any part of the body. Generalized vitiligo is the most common and usually
involves the face, lips, hands, arms, legs, and genital areas. Both sides of the body
are usually affected. Vitiligo can be psychologically devastating, especially when
present on visible areas of the body, such as the face and hands. The etiology of
vitiligo is unclear although it is believed to be an autoimmune disorder. The goal of
treatment is to restore the skin's color by restoring healthy melanocytes to the skin
(repigmentation) allowing the skin to regain its normal appearance. Individuals with
vitiligo should always protect their depigmented skin against excessive sun exposure
by wearing protective clothing, applying a UVA/UVB sunscreen daily, and avoid
prolonged sun exposure.
Treatments have highly variable results; therefore, treatment modalities should be
selected for maximum benefit and minimum risk, with consideration for the body
surface area involved. Topical corticosteroids are often used as first-line therapy.
Topical tacrolimus has shown efficacy in some trials and has the advantage over
topical corticosteroids as it does not cause skin atrophy. Tacrolimus often works best
when combined with NB-UVB light. Topical and oral psoralen ultraviolet A (PUVA)
and photochemotherapy or phototherapy with Ultraviolet A or B therapy have good
success rate. PUVA is effective for the face, trunk, upper arms, and upper legs. NBUVB requires two to three treatment sessions per week for several months.
Many studies of treatments for vitiligo are of poor quality and so evidence is limited,
particularly for the long-term benefits and safety of therapies. Welsh et al (2009)
evaluated the repigmentation response induced with broadband, UVB-targeted
phototherapy used as monotherapy in twelve patients with vitiligo affecting less than
10% of the skin surface. Patients were treated with 30 sessions of UVB-targeted
phototherapy administered twice weekly. The assessment of repigmentation was
made from a comparison of baseline photographs with those after 30 sessions by
two independent investigators. Morphometric analysis was performed using a
computer program. The authors reported that repigmentation with an average of
66.25% was obtained on lesions of the face, and of 31.5% on the neck, trunk, and
genitalia. On the extremities, there was no repigmentation. Itching, a burning
sensation, erythema, desquamation, and transitory hyperpigmentation were
observed in some patients. Minimal blistering and ulceration were observed in one
patient. They concluded that targeted UVB phototherapy seems to be effective for
the repigmentation of vitiligo in lesions located on the face, to a lesser degree on the
trunk, and with no response in acral lesions. Minimal adverse effects that did not
require discontinuation of treatment were reported.
A Cochrane review of all interventions for the treatment of vitiligo, reported by
Whitton et al (2008), identified nineteen randomized controlled trials. At least two
reviewers independently assessed study eligibility, methodological quality, and
extracted data. The authors concluded that variations in study design and different
outcome measures limit the evidence for the different therapeutic options. The best
evidence from individual trials showed short-term benefit from topical steroids and
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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various forms of UV light with topical preparations. They reported that long-term
follow-up and patient-centered outcomes should be incorporated in study design and
psychologic interventions need more attention.
Asawanonda et al (2008) compared the repigmenting efficacy of targeted broadband
UVB phototherapy with that of NB-UVB in a small number of patients with skin types
III, IV and V. Twenty identical vitiliginous lesions from 10 patients were randomly
allocated to receive either targeted broadband UVB or targeted NB-UVB
phototherapy. UV fluences were started at 50% of the minimal erythema dose
detected within the vitiliginous patches, then increased gradually, in the same
manner, to ensure equi-erythemogenic comparison. Treatments were carried out
twice weekly for 12 weeks. The results show that grade 1, i.e. 1-25%
repigmentation, to grade 2, 26-50% repigmentation, occurred in 6 of 10 subjects.
Responses in terms of repigmentation, de-pigmentation, or lack thereof, were similar
between lesions receiving broadband and NB-UVB phototherapy. Onset of
repigmentation occurred as early as 4 weeks of treatment in most subjects.
Treatments were well tolerated, with only minimal erythema and hyperpigmentation.
Bhatnagar et al (2007) compared the phototherapy modalities PUVA and NBUVB in
inducing stability in vitiligo, assessed by using vitiligo disease activity score (VIDA),
in an open prospective study of 50 individuals divided equally between the two
groups. In the NBUVB group, disease activity was present in 40% patients before
commencement of therapy, which was reduced to 16% at the end of therapy. In the
PUVA group, similar figures were 20% and 16%, respectively. In the NBUVB group,
50% of patients whose disease was active prior to commencement of therapy had
less than 50% repigmentation, whereas an equal number of patients had
repigmentation of more than 50%. Almost an equal number of stable patients had
less than and more than 50% repigmentation. In the PUVA group, 4 of the 5 (80%)
patients who had active disease had less than 50% repigmentation, whereas only 1
patient (20%) with active disease obtained more than 50% repigmentation. The time
to attain stability was 3.6 +/- 2.1 months in the NBUVB group and 3.22 +/- 3.1
months in the PUVA group. Eight of the 10 (80%) patients with unstable disease in
the NBUVB group achieved stability, whereas 2 of the 5 (40%) patients of similar
pre-treatment status in the PUVA group achieved stability.
Yones et al (2007) compared the efficacy of oral psoralen-UV-A (PUVA) with that of
narrowband-UV-B (NB-UVB) phototherapy in 56 patients with nonsegmental vitiligo.
The results in the 25 patients each in the PUVA and NB-UVB groups who began
therapy were analyzed. The median number of treatments was 47 in the PUVAtreated group and 97 in the NB-UVB-treated group. The author suggested that this
difference was because of the differences in efficacy and adverse effects between the
2 modalities, such that patients in the NB-UVB group wanted a longer course of
treatment. At the end of therapy, 16 (64%) of 25 patients in the NB-UVB group
showed greater than 50% improvement in body surface area affected compared with
9 (36%) of 25 patients in the PUVA group. The color match of the repigmented skin
was excellent in all patients in the NB-UVB group but in only 11 (44%) of those in
the PUVA group. In patients who completed 48 sessions, the improvement in body
surface area affected by vitiligo was greater with NB-UVB therapy than with PUVA
therapy. Twelve months after the cessation of therapy, the superiority of NB-UVB
tended to be maintained.
Scientific Rationale Initial
Phototherapy treatments used for specific skin conditions include all of the following:

Type A ultraviolet (UVA) radiation;
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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
Type B ultraviolet (UVB) radiation; and



UVB light can be categorized as wide-band and narrow-band, which
refers to the wavelengths included in the UV light source.
Phototherapy utilizes UVB treatments can also be combined with coal
tar applications, known as the Goeckerman regimen.
Combination UVA/UVB radiation.
Photochemotherapy includes psoralens (P) and type A ultraviolet (UVA) radiation,
known as PUVA photochemotherapy and combinations of P/UVA/UVB.

Photochemotherapy utilizes UVA in conjunction with a photosensitizer
called psoralen (also known as psoralen with Ultraviolet A, or PUVA for
short).

The photosensitizer known as psoralen is a medication that can be
applied directly to the skin or taken orally and makes the skin more
sensitive to the ultraviolet light.
Examples of phototherapy and photochemotherapy devices include, but may not be
limited to, Multiclear XL, ClearLight, Derma-Wand, Phototherapeutix, Daavlin
Ultraviolet Phototherapy Cabinet, TheraLight, and Lumenis BClear UVB Phototherapy
System.
Atopic Dermatitis (Eczema)
Dermatitis is as an inflammation of the skin and includes a wide variety of skin
disorders, including atopic dermatitis (eczema), seborrheic dermatitis, contact
dermatitis, latex dermatitis and allergy, and dyshidrotic dermatitis. Depending upon
the underlying cause, dermatitis can be a short-term or lifelong condition.
Eliminating factors that worsen eczema can effectively control the symptoms, in
most scenarios. Aggravating factors may include frequent bathing and dry
environments (which can further dry the skin), emotional stress, rapid temperature
changes, and exposure to certain chemicals and cleaning solutions. Common irritants
include soaps and detergents, perfumes and cosmetics, wool or synthetic fibers,
dust, sand, and cigarette smoke.
Ultraviolet light therapy or phototherapy can effectively control atopic dermatitis.
However, this therapy may increase a person's risk for skin cancer, and is therefore
recommended only for people with severe eczema who do not respond to
discontinuing the triggers noted above.
(2007) The National Institute for Clinical Excellence (NICE) published a report on the
treatment of atopic eczema in children up to age 12 years. The Guidance
Development Group concluded that phototherapy should only be considered for the
treatment of severe atopic eczema in children when other management options have
failed or are inappropriate and where there is a significant negative impact on quality
of life.
The American Academy of Dermatology (AAD) Practice Management "Guidelines of
Care for Atopic Dermatitis" includes phototherapy recommendations for atopic
dermatitis. These care guidelines are based upon a systematic review of literature
dated from 1990 to June 2003. Generally, the atopic dermatitis guidelines
recommend treatment with UV phototherapy, including combination BB-UVB/UVA,
nbUVB, PUVA and UVA (Hanifin, et al., 2004).
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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(2004) Guidelines developed by the Joint Task Force on Practice Parameters for
Allergy and Immunology, which is sponsored by the American Academy of Allergy,
Asthma, and Immunology, the American College of Allergy, Asthma, and
Immunology and the Joint Council of Allergy, Asthma and Immunology state:
“When atopic dermatitis is either severe or has not responded to appropriate
first-line management strategies, specialist consultation should be obtained. This
allows both a reevaluation of first-line treatment approaches (e.g., hydration,
emollients, topical corticosteroids, pimecrolimus, tacrolimus, and tar
preparations) and consideration of alternative therapy. Examples of alternative
strategies include (1) the application of wet dressings in combination with topical
corticosteroids; (2) short-term treatment with systemic corticosteroids with
appropriate tapering to avoid rebound; (3) phototherapy with ultraviolet light
(UV-B or UV-A [PUVA]); (4) immunomodulatory or immunosuppressive agents;
(5) hospitalization to separate the patient from environmental allergens while
administering other therapies; and (6) allergen immunotherapy when
aeroallergens are clearly implicated in dermatitis flares.”
Lichen planus
Lichen planus is a dermatological disease of unknown etiology affecting the skin and
oral mucous membranes, either alone or concomitantly. The oral lesions are most
common on the buccal mucous membrane. Histologically, lichen planus is
characterized by dense lymphocytic infiltrate at the dermal-epidermal junction. The
infiltrates consist of predominantly T cells, a finding suggesting the pathogenic role
of cell-mediated immunity.
The following treatment options for lichen planus are available:



Topical corticosteroids are helpful in patients with limited disease, and those
with oral lesions;
A 6- to 8-week course of oral corticosteroids is helpful in patients with
widespread lichen planus;
NB-UVB phototherapy or PUVA is helpful for widespread conditions.
Aydogan et al. (2008) Narrowband (NB-UVB) phototherapy has recently
demonstrated high levels of efficacy and tolerability in a variety of skin diseases. The
purpose of the present study was to assess the efficacy of NB-UVB phototherapy in
the management of pityriasis lichenoides (PL). The therapeutic response in 31 PL
patients treated with NB-UVB phototherapy between 2000 and 2007 was assessed.
NB-UVB treatment led to a complete response (CR) in 15 out of 23 individuals with
pityriasis lichenoides et varioliformis acuta (PLEVA) (65.2%) and a partial response
in eight patients (34.8%). NB-UVB treatment led to CR in seven out of eight patients
with pityriasis lichenoides chronica (PLC) (87.5%). Relapses occurred in four PL
patients within a mean time period of 6 months. NB-UVB therapy is an effective, safe
and practical alternative treatment modality for the management of PLEVA and PLC.
Photodermatoses (e.g., polymorphic light eruption, actinic prurigo, chronic
actinic dermatitis)
Photodermatoses indicate the development of cutaneous eruption secondary to
exposure to UV or visible radiation. UVB, UVA, and visible light are the relevant
spectra in photodermatoses.
Polymorphous light eruption is the most common idiopathic photodermatosis. Lesions
usually occur in early spring within a few hours of exposure to sunlight. Usually,
lesions persist for several days and resolve spontaneously. This chronic condition
tends to improve as the sunny season progresses, a phenomenon known as
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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“hardening.” Diagnosis is based on the typical history and morphologic features of
the lesion; the diagnosis can be confirmed by the induction of lesions with
provocative phototesting. When lesions occur primarily on the face, a diagnosis of
lupus must be excluded. Management consists of sun avoidance, the use of broadspectrum sunscreens, topical corticosteroids, and oral antihistamines. In severe
cases, desensitization treatment using NB-UVB or PUVA has been successful.
Desensitization is usually performed in early spring by exposing patients to
increasing doses of NB-UVB or PUVA.
Cutaneous T cell lymphoma (CTCL) (mycosis fungoides)
Cutaneous B-cell non-Hodgkin's lymphoma (NHL) represents approximately 10% of
all primary cutaneous lymphomas. Current initial therapies for CTCL are tailored to
the extent, burden, and type of disease present. Treatment include use of emollients
or topical corticosteroids, topical chemotherapy (nitrogen mustard, BCNU,
bexarotene), phototherapy, psoralen–ultraviolet A (PUVA) therapy, electron beam
irradiation, photon irradiation, extracorporeal photochemotherapy, chemotherapy,
peripheral blood stem cell transplantation, and allogeneic transplantation.
Mycosis fungoides is a variant of cutaneous T-cell lymphoma. The four types of
cutaneous manifestations are patch, plaque, tumor, and erythrodermic. The patches
are usually asymptomatic, although they occasionally may be mildly pruritic. As the
disease progresses, some of the patches may become more indurated and may
evolve into more elevated plaques. Nodular lesions may occur in patients without
any patch or plaque lesions, although more commonly these lesions occur in
conjunction with patches and plaques. Erythrodermic mycosis fungoides occurs as a
generalized erythroderma with significant scaling and pruritus. Therapy for mycosis
fungoides generally follows a sequential order: (1) topical nitrogen mustard or NBUVB, each of which can be combined with topical corticosteroids; (2) PUVA; (3)
topical retinoids daily, until lesions resolve; and (4) one of the following: oral
bexarotene or interferon-α subcutaneously 3 times weekly for 2 to 4 months.
Large Plaque Parapsoriasis
The two common variants of parapsoriasis are large plaque parapsoriasis and small
plaque parapsoriasis. In up to one third of patients, large plaque parapsoriasis may
evolve into mycosis fungoides. As a result, treatment of large plaque parapsoriasis is
similar to that of early-stage mycosis fungoides: high-potency topical corticosteroids,
topical nitrogen mustard, NB-UVB phototherapy, and PUVA. By comparison, patients
with small plaque parapsoriasis have a benign course, and management of small
plaque parapsoriasis should be symptomatic only, with emollients, topical
corticosteroids, and NB-UVB phototherapy.
Sclerotic Skin Disease (Connective Tissue Disease)
Connective tissue diseases, also referred to as sclerosing skin diseases, are a group
of clinical disorders that share an autoimmune etiology. Sclerosing skin diseases
include systemic sclerosis (SSc), localized scleroderma (LS) also known as morphea,
relapsing polychondritis, Sjogren syndrome, rheumatoid arthritis, adult-onset Still
disease, mixed connective tissue disease, dermatomyositis, sclerodermoid Cutaneous
Graft Versus Host Disease (GVHD), extragenital lichen sclerosus et atrophicus
(extragenital LSA), lupus erythematosus (LE), and sclerodermoid rarities (e.g.,
eosinophilic fasciitis, pansclerotic morphea [a severe variant of LS]), and POEMS
syndrome, which is characterized by polyneuropathy, organomegaly,
endocrinopathy, monoclonal gammopathy, and skin changes.
In systemic sclerosis, fibrosis of the skin can lead to considerable morbidity. No
significant improvement has been reported from studies investigating antifibrotic
therapies. Phototherapy with ultraviolet (UV) irradiation is successfully used for
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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treatment of several diseases because of its anti-inflammatory as well as
immunosuppressive mechanisms, and its low-risk profile. In addition, the UVA
spectrum in particular exerts antifibrotic effects as it leads to reduction of
procollagen synthesis and expression of collagenase-1 in vitro. Accordingly,
treatment with long-wavelength UVA-1 irradiation or photochemotherapy with UVA
plus the photosensitizer psoralen (PUVA) have been successfully used to reduce skin
fibrosis in localized scleroderma. Phototherapy has been shown to stop or inhibit the
fibrotic processes and to induce softening of sclerotic skin, especially in limited SSc.
Phototherapy thus represents a therapeutic alternative for antifibrotic treatment with
a low rate of adverse effects, which should be applied before the sclerotic process
has proceeded too far.
Review History
November 2008
January 2010
February 2010
March 2011
January 2012
January 2013
January 2014
January 2015
Medical Advisory Council Committee initial approval
Added treatment with phototherapy or topical or oral psoralen
ultraviolet A (PUVA) as medically necessary for the treatment
of vitiligo of the face and hands. Added link to the Vitiligo
policy.
Policy Updated. Treatment of vitiligo with ultraviolet B (UVB)
radiation (phototherapy) or topical or oral psoralen ultraviolet
A (photochemotherapy or PUVA) is no longer restricted to the
face and hands.
Update. Added Medicare Table. No revisions.
Update – no revisions
Update – no revisions. Added codes
Update – no revisions. Added codes
Update – no revisions. Codes updated
This policy is based on the following evidence-based guidelines:
1.
2.
3.
4.
5.
6.
7.
8.
Leung DY, Nicklas RA, Li JT, et al. Disease management of atopic dermatitis: an
updated practice parameter. Joint Task Force on Practice Parameters. Ann
Allergy Asthma Immunol 2004 Sep;93(3 Suppl 2):S1-21.
Hanifin JM, Cooper KD, Ho VC, et al. Guidelines of care for atopic dermatitis,
developed in Page 13 of 16. Coverage Position Number: 0031. accordance with
the American Academy of Dermatology (AAD)/American Academy of
Dermatology Association "Administrative Regulations for Evidence-Based Clinical
Practice Guidelines". J Am Acad Dermatol. 2004 Mar;50(3):391-404.
National Institute of Arthritis and Musculoskeletal and Skin Diseases. Vitiligo.
May 2001. Revised Oct. 2006. Available at:
http://www.niams.nih.gov/Health_Info/Vitiligo/default.asp#7
American Academy of Dermatology. Vitiligo.
Hayes. Search and Summary. Phototherapy for Atopic Dermatitis. December 29,
2010.
American Academy of Dermatology Work Group, Menter A, Korman NJ, et al.
Guidelines of care for the management of psoriasis and psoriatic arthritis:
section 6. Guidelines of care for the treatment of psoriasis and psoriatic arthritis:
case-based presentations and evidence-based conclusions. J Am Acad Dermatol.
2011 Jul;65(1):137-74.
Menter A, Korman NJ, Elmets CA, et al. Guidelines of care for the management
of psoriasis and psoriatic arthritis: Section 5. Guidelines of care for the
treatment of psoriasis with phototherapy and photochemotherapy. J Am Acad
Dermatol. 2010 Jan;62(1):114-35. Epub 2009 Oct 7.
Hayes. Medical Technology Directory. Phototherapy for Acne Vulgaris. February
13, 2009. Update February 23, 2012. Updated January 25, 2013. Updated March
13, 2014.
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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9.
Hayes. Medical Technology Directory. Ultraviolet B Phototherapy for Vitiligo.
February 26, 2010. Update February 21, 2012. Updated February 1, 2013.
Updated February 28, 2014.
10. Hayes. Health Technology Brief. Office-Based Phototherapy for Treatment of
Atopic Dermatitis in Adults. August 16, 2011. Update August 22, 2012. Updated
October 16, 2013. Archived September 16, 2014.
11. Hayes. Health Technology Brief. Office-Based Phototherapy for Treatment of
Atopic Dermatitis in Children. August 22, 2011. Archived August 22, 2012.
Updated October 16, 2013. Updated September 22, 2014.
12. Hayes. Health Technology Brief. Phototherapy for Early-Stage Mycosis
Fungoides. January 30, 2012. Updated January 16, 2014.
References – Update January 2015
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2.
3.
Chen X, Yang M, Cheng Y, et al. Narrow-band ultraviolet B phototherapy versus
broad-band ultraviolet B or psoralen-ultraviolet A photochemotherapy for
psoriasis. Cochrane Database Syst Rev. 2013.
Hoppe RT, Kim YK, Horowitz S. Treatment of early stage (IA to IIA) mycosis
fungoides. UpToDate. June 20, 2014.
Richard EG, Morrison W. Psoralen plus ultraviolet A (PUVA) photochemotherapy.
UpToDate. January 16, 2014.
References – Update January 2014
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2.
3.
Archier E, Devaux S, Castela E, et al. Efficacy of psoralen UV-A therapy vs.
narrowband UV-B therapy in chronic plaque psoriasis: A systematic literature
review. J Eur Acad Dermatol Venereol. 2012;26 Suppl 3:11-21.
Kim MB, Kim GW, Cho HH, et al. Narrowband UVB treatment of progressive
macular hypomelanosis. J Am Acad Dermatol. 2012;66(4):598-605.
Weberschock T, Strametz R, Lorenz M, et al. Interventions for mycosis
fungoides. Cochrane Database Syst Rev. 2012;9
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4.
5.
Kerr AC, Ferguson J, Attili SK, et al. Ultraviolet A1 phototherapy: a British
Photodermatology Group workshop report. Clin Exp Dermatol. 2012 Apr; 37
(3):219-26. Epub 2012 Jan 25.
Kreutz M, Karrer S, Hoffmann P, et al. Whole-body UVB irradiation during
allogeneic hematopoietic cell transplantation is safe and decreases acute graftversus-host disease. J Invest Dermatol. 2012;132(1):179-187.
Reynolds NJ, Franklin V, Gray JC, et al. Narrow-band ultraviolet B and broadband ultraviolet A phototherapy in adult atopic eczema: A randomised controlled
trial. Lancet. 2001;357(9273):2012-2016.
Walker D, Jacobe H. Phototherapy in the age of biologics. Semin Cutan Med
Surg. 2011 Dec;30(4):190-8. .
Zandi S, Kalia S, Lui H. UVA1 phototherapy: a concise and practical review. Skin
Therapy Lett. 2012 Jan;17(1):1-4.
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Babilas P, Szeimies RM. The use of photodynamic therapy in dermatology. G Ital
Dermatol Venereol. 2010 Oct;145(5):613-30.
Dogra S, De D. Phototherapy and photochemotherapy in childhood dermatoses.
Indian J Dermatol Venereol Leprol. 2010 Sep-Oct;76(5):521-6
References – Update March 2011
1.
Menter A, Korman NJ, Elmets CA, et al. Guidelines of care for the management
of psoriasis and psoriatic arthritis: Section 5. Guidelines of care for the
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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2.
treatment of psoriasis with phototherapy and photochemotherapy. J Am Acad
Dermatol. 2010;62(1):114-135.
Tzaneva S, Kittler H, Holzer G, et al. 5-Methoxypsoralen plus ultraviolet (UV) A
is superior to medium-dose UVA1 in the treatment of severe atopic dermatitis: A
randomized crossover trial. British Journal of Dermatology. 162 (3) (pp 655660), 2010.
References – Update January 2010
1. Abdulla SJ, Desgroseilliers JP. Treatment of vitiligo with narrow-band ultraviolet
B: advantages and disadvantages. J Cutan Med Surg. 2008 Jul-Aug;12(4):1749.
2. Akar A, Tunca M, Koc E, Kurumlu Z. Broadband targeted UVB phototherapy for
localized vitiligo: a retrospective study. Photodermatol Photoimmunol Photomed.
2009 Jun;25(3):161-3.
3. Asawanonda P, Kijluakiat J, Korkij W, Sindhupak W. Targeted broadband
ultraviolet b phototherapy produces similar responses to targeted narrowband
ultraviolet B phototherapy for vitiligo: a randomized, double-blind study. Acta
Derm Venereol. 2008;88(4):376-81.
4. Bhatnagar A, Kanwar AJ, Parsad D, De D. Comparison of systemic PUVA and NBUVB in the treatment of vitiligo: an open prospective study. J Eur Acad Dermatol
Venereol. 2007 May;21(5):638-42.
5. Bhatnagar A, Kanwar AJ, Parsad D, De D. Psoralen and ultraviolet A and narrowband ultraviolet B in inducing stability in vitiligo, assessed by vitiligo disease
activity score: an open prospective comparative study. J Eur Acad Dermatol
Venereol. 2007 Nov;21(10):1381-5.
6. Brazzelli V, Antoninetti M, Palazzini S, et al. Critical evaluation of the variants
influencing the clinical response of vitiligo: study of 60 cases treated with
ultraviolet B narrow-band phototherapy. J Eur Acad Dermatol Venereol. 2007
Nov;21(10):1369-74.
7. Forschner T, Buchholtz S, Stockfleth E. Current state of vitiligo therapy-evidence-based analysis of the literature. J Dtsch Dermatol Ges. 2007
Jun;5(6):467-75
8. Percivalle S, Piccino R, Caccialanza M, Forti S. Narrowband UVB phototherapy in
vitiligo: evaluation of results in 53 patients. G Ital Dermatol Venereol. 2008
Feb;143(1):9-14
9. Welsh O, Herz-Ruelas ME, Gómez M, Ocampo-Candiani J. Therapeutic evaluation
of UVB-targeted phototherapy in vitiligo that affects less than 10% of the body
surface area. Int J Dermatol. 2009 May;48(5):529-34.
10. Whitton ME, Ashcroft DM, González U. Therapeutic interventions for vitiligo. J
Am Acad Dermatol. 2008 Oct;59(4):713-7
11. Yones SS, Palmer RA, Garibaldinos TM, Hawk JL. Randomized double-blind trial
of treatment of vitiligo: efficacy of psoralen-UV-A therapy vs Narrowband-UV-B
therapy. Arch Dermatol. 2007 May;143(5):578-84.
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Ong PY, Bogienewitz M. Atopic Dermatitis. Primary Care: Clinics in Office
Practice. Volume 35, Issue 1 (March 2008).
Sunderkötter C, Kuhn A, Hunzelmann N, et al. Phototherapy: a promising
treatment option for skin sclerosis in scleroderma? Rheumatology (Oxford). 2008
Feb; 47(2): 234-5.
Habermann TM, Pittelkow MR. Chapter 113, Cutaneous T-Cell Lymphoma and
Cutaneous B-Cell Lymphoma. Abeloff: Abeloff's Clinical Oncology, 4th ed. 2008.
Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01)
phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed.
2008 Jun;24(3):128-33.
Phototherapy and Photochemotherapy (PUVA) for Dermatological Conditions Jan 15
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treatment with UVA-1 in sclerodermic skin diseases. J Eur Acad Dermatol
Venereol. 2008 Jul;22(7):839-44. Epub 2008 Apr 30.
Rombold S, Lobisch K, Katzer, et al. Efficacy of UVA1 phototherapy in 230
patients with various skin diseases. Photodermatol Photoimmunol Photomed.
2008 Feb;24(1):19-23.
Wang F, Garza LA, Cho S, et al. Effect of Increased Pigmentation on the
Antifibrotic Response of Human Skin to UV-A1 Phototherapy. Arch Dermatol.
2008; 144 (7): 851-858.
Weston WL, Howe W. Dermatitis. UpToDate. 2007.
Wackernagel A, Legat FJ, Hofer A, et al. Psoralen plus UVA vs. UVB-311 nm for
the treatment of lichen planus. Photodermatol Photoimmunol Photomed. 2007
Feb;23 (1):15-9.
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report 1998-2006. Determining the potential to improve quality of care: 8th
edition. ULTIMO NSW: Australian Council on Healthcare Standards (ACHS);
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psoriatic arthritis. 2007.
Clayton TH, Clark SM, Turner D, Goulden V. The treatment of severe atopic
dermatitis in childhood with narrowband ultraviolet B phototherapy. Clin Exp
Dermatol. 2007 Jan;32 1):28-33.
Kirke SM, Lowder S, Lloyd JJ, Diffey BL, Matthews JN, Farr PM. A randomized
comparison of selective broadband UVB and narrowband UVB in the treatment of
psoriasis. J Invest Dermatol.2007 Jul;127(7):1641-6. Epub 2007 Mar 22.
Legat FJ, Hofer A, Wackernagel et al. Narrowband UV-B phototherapy, alefacept,
and clearance of psoriasis. Arch Dermatol. 2007 Aug;143(8):1016-22.
Meduri NB, Vandergriff T, Rasmussen H, Jacobe H. Phototherapy in the
management of atopic dermatitis: a systematic review. Photodermatol
Photoimmunol Photomed. 2007 Aug;23(4):106-12.
Brown S, Reynolds NJ. Atopic and non-atopic eczema. BMJ. 2006 Mar
11;332(7541):584-8.
El-Mofty M, Mostafa W, Youssef R, et al. Nonlaser UVB-targeted phototherapy
treatment of psoriasis. Cutis. 2006 Sep;78(3):200-3.
Koek MB, Buskens E, Bruijnzeel-Koomen CA, Sigurdsson V. Home ultraviolet B
phototherapy for psoriasis: Discrepancy between literature, guidelines, general
opinions and actual use. Results of a literature review, a web search, and a
questionnaire among dermatologists. Br J Dermatol. 2006;154(4):701-711.
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