Cutaneous Lupus Erythematosus: Current Treatment Options
Curr Treat Options in Rheum
Cutaneous Lupus Erythematosus: Current Treatment Options
Jenna K. Presto
Victoria P. Werth 0
0 Corporal Michael J. Crescenz Veterans Affairs Medical Center (Philadelphia) , Philadelphia, PA , USA
Cutaneous lupus erythematosus I Topical steroids I Topical calcineurin inhibitors I Antimalarials I; Immunosuppressives I Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI™)
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Opinion statement
Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune skin disease that can
present with or without systemic lupus erythematosus (SLE). Managing CLE is important in
order to reduce potential and established areas of damage, as well as improve quality of life
(QOL). Non-drug therapy should be used in every case, which includes photoprotection,
smoking cessation, and avoiding drugs that can trigger or exacerbate the disease. Topical
corticosteroids and topical calcineurin inhibitors are often used in addition to lifestyle
changes. Antimalarials are first-line systemic therapies, with hydroxychloroquine being the
drug of choice. Quinacrine can be added to hydroxychloroquine for greater efficacy in
hydroxychloroquine-refractory patients, and chloroquine can be used in place of
hydroxychloroquine in patients who are unable to tolerate hydroxychloroquine. Second-line
therapies include oral retinoids, immunosuppressives, immunomodulators, biologics, and
pulsed dye laser. Systemic steroids may be necessary when bridging therapies but should
normally be avoided due to their side effects. There is a paucity of high quality evidence with
regard to management of cutaneous lupus, making it challenging to determine an appropriate
treatment in refractory cases. Trials on existing therapies as well as new therapies are
necessary in order to better treat patients with CLE.
Introduction
Lupus erythematosus (LE) is a broad-spectrum disease
which can manifest as cutaneous lupus erythematosus
(CLE) or multisystem organ disease with systemic lupus
erythematosus (SLE). CLE is divided into subgroups,
Cutaneous Lupus Erythematosus: Current Treatment Options
consisting of chronic cutaneous lupus erythematosus
(CCLE), subacute cutaneous lupus erythematosus
(SCLE), and acute lupus erythematosus (ACLE). CCLE
can be further divided; discoid lupus erythematosus
(DLE) and lupus erythematosus tumidus (LET) are the
most common forms of CCLE, while lupus panniculitis,
hypertrophic lupus, mucosal lupus, chilblain lupus, and
lichenoid DLE are less common forms of CCLE.
The goal of cutaneous lupus treatment is to reduce
potential and established areas of activity and damage.
The Cutaneous Lupus Erythematosus Disease Area and
Severity Index (CLASI™) is a scoring system that has been
validated to assess for skin disease activity and damage
in cutaneous lupus patients. It has demonstrated
intrarater and interrater reliability in dermatologists
and rheumatologists [1, 2]. This scoring system has been
used in numerous trials and has been shown to correlate
with quality of life measures and disease improvement
with a 4-point or 20 % decrease in CLASI™ activity score
deemed clinically significant [2, 3].
Improving quality of life is an important factor in
treatment. Quality of life in cutaneous lupus patients
has been assessed using the Skindex 29 + 3 [4], a
Lifestyle
validated skin-specific quality of life instrument, and
the SF-36 [5], a validated general health quality of life
form [6]. Patients with cutaneous lupus have a worse
quality of life (QOL) as compared to other dermatologic
conditions, such as acne, non-melanoma skin cancer,
and alopecia. Poor quality of life for CLE patients has
been associated with female gender, generalized disease,
severe disease, facial lesions, and younger age [7]. These
QOL indicators have been found to be similar between
geographic populations [8]. Antimalarial or
antimetabolite treatment response leads to decreased emotion,
functioning, and symptom score using the Skindex 29
[9 ]. This suggests the impact of treatment on a patient’s
well-being.
Treatment of cutaneous lupus should be based on
severity and type of cutaneous lupus. Prevention of new
lesions is the first key step. Prevention includes
photoprotection, smoking cessation, and removal of
exacerbating drugs. Topical therapy is often initiated
with these preventative measures. Antimalarials remain
the first-line systemic therapy and other systemic agents
are used for recalcitrant cases or in patients who are
unable to tolerate the antimalarials.
UVA and UVB irradiation has been shown to induce lesions in patients with
cutaneous lupus [
10
]. It is suggested that UV protection can reduce UV-triggered
CLE lesions by inhibiting an interferon-mediated inflammatory response.
CD11c- and CD123-positive dendritic cells, a source of interferons (IFNs), as
well as IFN-induced human protein MxA are increased in unprotected
irradiated skin of CLE patients and decreased when protected by broad spectrum
sunscreen [ (...truncated)