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Efficacy and safety of diphenylcyclopropenone among Chinese patients with steroid resistant and extensive alopecia areata

Luk NM, Chiu LS, Lee KC, Chau CT, Lee VW, Chang M, et al.

J Eur Acad Dermatol Venereol, 2013 Mar;27(3):e400-5.
PMID: 23057682 DOI: 10.1111/jdv.12009

BACKGROUND: Topical immunotherapy has recently been found useful in the treatment of chronic and extensive Alopecia Areata (AA).
OBJECTIVE: To evaluate the efficacy and safety of diphenylcyclopropenone (DPCP) use among Chinese patients with steroid resistant and extensive AA in our institute.
METHODS: The medical records of 31 Chinese patients treated with DPCP were analysed retrospectively. The efficacy, adverse effects, and relapse rate of DPCP treatment were reviewed.
RESULTS: Thirty-one (16 male, 15 female) Chinese patients with extensive, steroid resistant Alopecia Areata and a mean age of 28.9 years (SE 10.4) were treated. The mean age of onset was 17.8 years (SE 8.8) with an average disease duration of 11.2 years (SE 7.7). Ten patients had a history of atopy and 4 had a history of thyroid disease. Nail changes were found in 14 patients and a family history of AA was found in 2 patients. Thirteen patients (41.9%) had experienced total hair loss. Two patients abandoned the treatment due to severe side effects. Of the remaining 29 patients, 4 (13.8%), 7 (24.1%), 5 (17.2%), and 13 (44.8%) achieved >90% complete response, >50-90% partial response, >10-50% minimal response, and <10% no response hair regrowth, respectively. Adverse effects included pruritus, erythema, vesiculation, scaling, cervical lymphadenopathy, dyspigmentation and urticarial reactions. Relapse occurred (>25% hair loss) in 69.23% of patients after 18 months of follow up.
CONCLUSIONS: DPCP is an effective and tolerable treatment for Chinese patients with extensive, steroid resistant AA.
Skin disease profile of Syrian refugees in Jordan: a field-mission assessment

Saikal SL, Ge L, Mir A, Pace J, Abdulla H, Leong KF, et al.

J Eur Acad Dermatol Venereol, 2020 Feb;34(2):419-425.
PMID: 31498503 DOI: 10.1111/jdv.15909

BACKGROUND: Since the beginning of the Syrian war in 2011, the world has faced the most severe refugee crisis in history and 5.6 million Syrians have sought asylum in neighbouring countries or in Europe. According to recent estimates, more than 650 000 Syrian refugees are displaced in Jordan.
OBJECTIVES: This article aims to assess the demographic characteristics and skin disease profile of Syrian displaced people residing in Al Za'atari camp and in communities in Jordan. Furthermore, the authors discuss the barriers to healthcare provision experienced during field missions.
METHODS: This is a retrospective analysis of medical records collected during three medical missions in Jordan by an international dermatological team. Data on patient age, gender, country of origin and skin disease diagnoses were recorded both in Al Za'atari camp and Jordanian towns near the Syrian border.
RESULTS: A total of 1197 patients were assessed during the field missions, with 67.7% female and 37.1% under the age of 14 years. Dermatitis was the leading dermatological condition in both refugee camp and community healthcare clinics. Infectious diseases were the second most common; however, fungal presentations were more common in the community as opposed to viral in Al Za'atari.
CONCLUSIONS: High dermatitis presentations were likely secondary to the environment, living conditions and lack of access to emollients. Infectious diseases were postulated secondary to poor hygiene and sharing of overcrowded spaces. Barriers to health care included limited pharmacological formulary, difficulty in continuity of care and case referrals due to lack of specialized services. Better access to health care, improvement of living conditions and hygiene, and increased availability of medications including emollients and sunscreens are all interventions that should be carried out to reduce skin disease burden. Our findings should further urge the international community to uphold their commitments and uptake engagement in improving health care for Syrian displaced people.
Generalized pustular psoriasis: A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management

Puig L, Choon SE, Gottlieb AB, Marrakchi S, Prinz JC, Romiti R, et al.

J Eur Acad Dermatol Venereol, 2023 Apr;37(4):737-752.
PMID: 36606566 DOI: 10.1111/jdv.18851

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and highly heterogeneous skin disease, characterized by flares of neutrophilic pustules and erythema. As a rare disease with few clinical studies and no standardized management approaches, there is a paucity of knowledge regarding GPP.
OBJECTIVES: Conduct a Delphi panel study to identify current evidence and gain advanced insights into GPP.
METHODS: A systematic literature review was used to identify published literature and develop statements categorized into four key domains: clinical course and flare definition; diagnosis; treatment goals; and holistic management. Statements were rated on a Likert scale by a panel of dermatologists in two rounds of online questionnaires; the threshold for consensus was agreement by ≥80%.
RESULTS: Twenty-one panellists reached consensus on 70.9%, 61.8%, 100.0% and 81.8% of statements in the 'clinical course and flare definition', 'diagnosis', 'treatment goals' and 'holistic management of GPP' domains, respectively. There was clear consensus on GPP being phenotypically, genetically and immunologically distinct from plaque psoriasis. Clinical course is highly variable, with an extensive range of complications. Clinical and histologic features supporting GPP diagnosis reached high levels of agreement, and although laboratory evaluations were considered helpful for diagnosis and monitoring disease severity, there was uncertainty around the value of individual tests. All acute and long-term treatment goals reached consensus, including rapid and sustained clearance of pustules, erythema, scaling and crust, clearance of skin lesions and prevention of new flares. Potential triggers, associated comorbidities and differential diagnoses achieved low rates of consensus, indicating that further evidence is needed.
CONCLUSIONS: Global consensus between dermatologists was reached on clinically meaningful goals for GPP treatment, on key features of GPP flares and on approaches for assessing disease severity and multidisciplinary management of patients. On this basis, we present a management algorithm for patients with GPP for use in clinical practice.
Prevalence, comorbidities and mortality of generalized pustular psoriasis: A literature review

Prinz JC, Choon SE, Griffiths CEM, Merola JF, Morita A, Ashcroft DM, et al.

J Eur Acad Dermatol Venereol, 2023 Feb;37(2):256-273.
PMID: 36331364 DOI: 10.1111/jdv.18720

Generalized pustular psoriasis (GPP) is a rare auto-inflammatory skin disease characterised by acute episodes of sterile pustule formation. Diagnosis and treatment of the disease have historically been complicated by a lack of awareness, and no consistent global definition or clinical coding standards. Now acknowledged as a distinct clinical entity with a recognised genetic component, GPP can take a serious and life-threatening course due to systemic inflammatory complications and its association with various comorbidities. As with other rare diseases, there are significant challenges to understanding the epidemiology of GPP, notably a small patient population, non-standardised study methodologies and ethnic differences in its presentation. A clearer understanding of GPP is therefore required for clinicians to better manage patients with this rare condition. In this review article, we present an overview of the available data on GPP prevalence estimates in key demographics and report the frequency of genetic mutations associated with the disease. We detail the incidence of known comorbidities and summarise the data on mortality and assigned causes of death. Lastly, we discuss the various factors that impact the collection, interpretation and comparison of these data.
Health-related quality of life in patients with generalized pustular psoriasis: A systematic literature review

Choon SE, De La Cruz C, Wolf P, Jha RK, Fischer KI, Goncalves-Bradley DC, et al.

J Eur Acad Dermatol Venereol, 2024 Feb;38(2):265-280.
PMID: 37750484 DOI: 10.1111/jdv.19530

Generalized pustular psoriasis (GPP) is a rare, chronic, neutrophilic inflammatory skin disease characterized by episodes of widespread eruption of sterile, macroscopic pustules that can be accompanied by systemic inflammation and symptoms. A systematic literature review and narrative synthesis were conducted to determine the impact of GPP on patients' health-related quality of life (HRQoL) and patient-reported severity of symptoms and to compare its impact to patients with plaque psoriasis (plaque PsO). Searches were undertaken in Embase, MEDLINE and the Cochrane Library from 1 January 2002 to 15 September 2022. Screening was carried out by two reviewers independently. Outcome measures included generic (e.g. EQ-5D, SF-36) and dermatology-specific (e.g. DLQI) clinical outcome assessments, and other relevant patient-reported outcome measures (PROMs) (e.g. severity of pain measured by a numerical rating scale). Overall, 20 studies were found to be eligible for inclusion, of which seven also had data for plaque PsO. The DLQI was the most frequently reported outcome measure (16 out of 20 studies). When reported, mean DLQI (SD) scores varied from 5.7 (1.2) to 15.8 (9.6) across the studies, indicating a moderate to very large effect on HRQoL; the wide range of scores and large SDs were explained by the small population sizes (n ≤ 12 for all studies except two). Similar ranges and large SDs were also observed for other measures within individual studies. However, in general, people with GPP reported a greater impact of their skin condition on HRQoL, when compared to people with plaque PsO (i.e. higher DLQI scores) and higher severity for itch, pain and fatigue. This systematic review highlighted the need for studies with a larger population size, a better understanding of the impact of cutaneous and extracutaneous symptoms and comorbidities on HRQoL during and between GPP flares, and outcome measures specifically tailored to the unique symptoms and the natural course/history of GPP.