Acta Dermato-Venereologica 99-10CompleteContent | Page 18
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Dupilumab in Treatment of Chronic Prurigo: A Case Series and Literature Review
Zaid Z. ALMUSTAFA, Karsten WELLER, Juliane AUTENRIETH, Marcus MAURER and Martin METZ*
Department of Dermatology and Allergy, Charité – Universitätsmedizin Berlin, Charitéplatz 1, DE-10117 Berlin, Germany. *E-mail: martin.
[email protected]
Accepted Jun 18, 2019; E-published Jun 24, 2019
Chronic prurigo (CPG) is a common and distinct skin
disease defined by the presence of chronic pruritus,
signs of repeated scratching and multiple pruriginous
skin lesions (1). A large proportion of patients with CPG
have dramatically impaired quality of life and are largely
resistant to available therapy.
The pathophysiological mechanisms leading to CPG
are currently unknown. Atopy is thought to play an
important role, with approximately half of all patients
with CPG having an atopic disposition (1). Furthermore,
atopic dermatitis (AD) is the most frequent skin disease
associated with CPG (2). It is important to note, however,
that CPG is a distinct disease, which can evolve on the
basis of an AD or other inflammatory skin disease, as
well as various systemic disorders, and then co-exist or
continue after their cessation (3). In most cases, CPG is
thought to have a multifactorial or undetermined origin
(4). Currently, there are no approved therapies available.
Most patients receive insufficient therapies, consisting
of topical anti-inflammatory treatments and systemic
H1 antihistamines. Some patients are treated by specia-
list centres with off-label drugs, such as cyclosporine,
anticonvulsants, μ-opioid- or neurokinin-1-receptor
antagonists (5).
In many patients with CPG an atopic disposition can be
observed, leading to a Th2-driven immune dysregulation.
Furthermore, in many patients with CPG a pronounced
superficial, perivascular and/or interstitial inflammatory
infiltrate, composed mainly of lymphocytes, macropha-
ges, and eosinophils, can be identified in lesional skin (6).
The human mAb dupilumab, a monoclonal anti-IL-4Rα
antibody, acts on both features. In AD, dupilumab has
been shown to lead to a significant reduction in disease
activity, as well as to a rapid, significant and clinically
relevant improvement in pruritus (7). Based on these
A
SHORT COMMUNICATION
B
Itch intensity
10 during day
8 during night
DLQI
30
very large effect
4
10
2
0
Baseline 2
4
6
8
10
Time after treatment initiation
12
0
moderate
small
Baseline
4
6
CASE REPORTS
Patient 1. A 41-year-old man was diagnosed with nodular type
CPG in 2016. The patient had a history of allergic asthma and mul-
tiple type I allergies, highly elevated total IgE of 14,778 kU/I and,
at the time of first consultation, generalized eczema. At this time,
the patient was diagnosed with AD, which was well controlled by
ultraviolet B (UVB) 311 nm phototherapy and topical steroids.
However, the patient continued to report extremely troublesome
pruritus, along with continuous development of new CPG nodules.
Different therapeutic approaches (see Table SI 1 ) were unsuccessful,
and the patient continued to experience severe pruritus. In Decem-
ber 2017, the patient reported constant pruritus with a maximum
intensity of 10 on a numerical rating scale (NRS, 0–10), a major
impairment in quality of life (Dermatology Life Quality Index
(DLQI) score of 25, ItchyQoL Score of 88), and presented with
numerous highly pruritic nodules and plaques. As cyclosporine
treatment was not possible due to poorly controlled hypertension,
treatment with dupilumab was initiated. The patient reported a
significant improvement in symptoms 2 weeks after receiving
the loading dose of 600 mg (Fig. 1). His condition continued to
improve, and from week 6 after the start of dupilumab the patient
reported a dramatic improvement in pruritus and prurigo control
(Fig. 1A, D), only mild impairment in quality of life (Fig. 1B, C),
and a slow, but steady, improvement in CPG lesions.
Patient 2. A 45-year-old woman was diagnosed with CPG of
nodular type in 2015, with multiple chronic and extremely itchy
excoriated nodules on her legs, buttocks and arms. The patient had
an atopic history, including multiple type 1 allergies, dyshidrotic
hand eczema, and allergic asthma. Despite numerous therapeutic
attempts (Table SI 1 ), the patient reported constant pruritus, with a
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3243
1
ItchyQoL
C
extremely large effect
on patient's life
20
6
findings, dupilumab may be a beneficial treatment option
for patients with CPG.
We report here 3 patients with CPG who were treated
with dupilumab in our department within the last 12
months.
8
10
12
Time after treatment initiation (weeks)
110
100
90
80
70
60
50
40
30
20
10
0
D
Prurigo Control Test
20
severe
15
moderate
10
mild
5
little
Baseline 2
4
6
8
10
12
Time after treatment initiation
0
Baseline 2
4
6
8
10
12
Time after treatment initiation (weeks)
Fig. 1. Improvement in pruritus and quality of life during dupilumab treatment in a patient with chronic prurigo. (A) Maximum itch intensity
during day and night. Dermatological and itch-specific quality of life impairment (DLQI, B; ItchyQoL, C), and level of disease control of chronic prurigo
(Prurigo Control Test, D) were assessed during the patients’ visits every 2–4 weeks for up to 12 weeks. The patient has now been under treatment for
> 9 months and shows a sustained response to dupilumab.
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3243
Acta Derm Venereol 2019; 99: 905–906