The autosomal recessive form of the Hyper IgE syndrome (AR-HIES) with dedicator of cytokinesis 8 (DOCK8) deficiency is associated with difficult to treat persistent viral skin infections, including papilloma virus infection. Type I interferons play an important role in the defense against viruses. We examined the effect of therapy with IFN–α 2b in an 11-year old boy with DOCK8 deficiency due to a homozygous splice donor site mutation in DOCK8 intron 40. His unremitting warts showed dramatic response to IFN–α 2b therapy. Immunological studies revealed decreased circulating plasmacytoid dendritic cells (pDCs) and profound deficiency of IFN-α production by his peripheral blood mononuclear cells in response to treatment with CpG oligonucleotides. These findings indicate that underlying pDC deficiency and impaired IFN-α production may predispose to chronic viral infections in DOCk8 deficiency. IFN–α 2b therapy maybe useful in controlling recalcitrant viral infections in these patients.
Background: Psoriasis vulgaris is a common chronic dermatological disease that has a negative impact on the psychological status and the social interaction of the patient.Objective: To correlate the quality of life and clinical severity of psoriasis vulgaris in patients using different types of treatment. Materials and methods: This is a prospective observational cross-sectional study that took place over a 4 month period, from January to April 2014, at the King Abdul Aziz Medical City, Jeddah, Saudi Arabia. All patients with psoriasis vulgaris attending the dermatology clinics during this period were included.Results: Of the total 41 Saudi patients, 25 were males and 16 were females. 21 were on combined (i.e. topical with either systemic or NB-UVB) and 20 were on topical treatments only. PASI score was moderate in the majority for both groups, that is, patients on topical (70%) and combined treatment (80%). DLQI score shows only a small effect on the patient's quality of life for the majority (14/20) in the topical group and (16/21) for those on combined therapy.Conclusion: There is no significant difference in terms of the effect of the type of treatment between the two groups. Ó 2015 The Authors. Production and hosting by Elsevier B.V. on behalf of King Saud University. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
. Jeddah is the major city in the Western region, with an approximate population of two million. The city is located on the Red Sea, and has a hot climate, with daily temperatures ranging from 15-43°C. throughout the year, with high humidity at times and only occasional rainfall. The King Khalid National Guard Hospital is one of the major government hospitals in the region catering for national guard military personnel and their families, as well as non-national guard patients. The hospital offers secondary and tertiary care, and is equipped with modern sophisticated facilities. Materials and MethodsThis was a prospective study that included all new patients attending the Skin Clinic at King Khalid National Guard Hospital during the study period. All patients were seen by consultants and specialists. The diagnosis in each case was based on clinical findings, and histopathological examination of skin biopsies where necessary. The diseases were classified into nine major groups, according to the ninth revision of the International Classification of Diseases (ICD-9). ResultsDuring the 12-month study period, the total number of patients attending the Dermatology and Venereology Outpatient Clinic at the hospital was 5260. Of this number, 910 (17.3%) were new patients. There were 782 adult (>12 years) patients (85.93%) and 128 children (<12 years) (13.79%). There were 418 males (45.93%) and 492 females (54.06%), with a female/male ratio of 1.2:1. Saudi nationals constituted the majority of patients at 860 (94.5%) with non-Saudis numbering only 50 (5.49%). National Guard personnel comprised 149 (16.36%), their families 367 (40.33%), and non-National Guard patients were 394 (43.29%).A total of 928 new diagnoses were seen during the study period. These were classified into nine categories according to the frequency of presentation. Table 1 shows the main disease groups, of which eczema/dermatitis topped the list (18.64%), followed by acne (9.48%), viral infections (9.26%), bacterial infections (7.65%), pigmentary disorders (6.46%), fungal infections (6.35%), papulosquamous diseases (4.84%), and urticaria (2.37%). Miscellaneous skin conditions were diagnosed in the remaining 328 patients (34.9%), and disorders of hair and pruritus were the most common. DiscussionThe current study is the first to describe the patterns of skin diseases in the Western region of the Kingdom of Saudi Arabia. The King Khalid Hospital is a referral center for primary health clinics in Jeddah, Makkah and Taif. Although this study was limited to our hospital, we believe that the results represent a rough estimate of the prevalence of skin diseases in the Western region, and that the distribution of dermatoses according to age and sex reflect the true prevalence (as both the referral and the eligibility systems of the hospital apply no specific restrictions).
The current treatment of psoriasis patients with biologic agents in the Kingdom of Saudi Arabia (KSA) is mainly based on clinical experience. Although there are published international guidelines for treatment with biologics, such as the European S3 guidelines (a joint project of the European Dermatology Forum, the European Academy of Dermatology and Venereology, and the International Psoriasis Council), many nations have found it beneficial to develop country-based guidelines that incorporate specific regional aspects of therapy (legal and practical). With the expanded role of biologic agents in the treatment of psoriasis in Saudi Arabia, a need for local Saudi guidelines has become evident. Here we present a practical approach to the evidence-based clinical administration of biologics for professionals who treat patients with psoriasis.
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