Acta Dermato-Venereologica 99-10CompleteContent | Page 18

905 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