Implementation and evaluation of an algorithm for the management of scabies outbreaks.

BACKGROUND: Infestations with scabies mites are a global burden affecting individuals of all ages, classes and ethnicities. As poor sanitation and overcrowding favor the transmission of this highly contagious disease, epidemic outbreaks are frequently observed among displaced persons and asylum seekers. Due to the growing influx of refugees during the last years, public health authorities in host countries are frequently confronted with the challenge to treat individuals with diagnosed or suspected scabies promptly and effectively to avoid further spreading of the infestation. This study aimed to establish a straightforward and efficient algorithm for rapid screening and treatment of large numbers of patients with confirmed or suspected scabies infestations. METHODS: Forty-eight individuals (58% males, mean age 22.4 yrs.) from Syria with suspected scabies infestation were allocated to 3 colour-coded groups: (1) no signs or symptoms of infestation, (2) itch only, and (3) itch and typical skin lesions. Patients were treated with a single (group 1) or two doses of oral ivermectin at an interval of 7 days (group 2), or with a combination of 2 doses of ivermectin plus 2 applications of permethrin ointment at an interval of 7 days (group 3). Follow-ups were performed 4 weeks after initial treatments. RESULTS: All individuals with signs and/or symptoms of infestation had improved skin lesion; in 10/11 (90.9%) lesion had completely resolved. All individuals with initial itch only (n = 32) reported improvement of its intensity or complete resolution. None of the patients of group 1 developed itch or skin lesions. The algorithm was reapplied in 4 individuals (8.3%) after 4 weeks and the outbreak was completely controlled after 8 weeks. Colour-coding ensured fast flow of information between health-care providers at the interfaces of the algorithm. CONCLUSIONS: Our algorithm proved to be both highly efficient for treatment of large numbers of patients with suspected or diagnosed scabies infestation as well as for prevention of spreading of the disease. Hence, this algorithm is well suited for the management of scabies mass outbreaks.

Genital chronic GVHD in men after hematopoietic stem cell transplantation: a single-center cross-sectional analysis of 155 patients.

We assessed the prevalence and clinical features of genital skin changes in men after allogeneic hematopoietic stem cell transplantation (HSCT) and evaluated the correlation between genital chronic graft-versus-host disease (cGVHD) and other manifestations of cGVHD as well as sexual issues. In a cross-sectional cohort study, 155 male recipients alive 1 year or more after HSCT were assessed during their annual follow-up evaluation. Correlation between genital skin changes and other cGVHD manifestations was evaluated, and post-transplantation sexual contentment and sexual functioning were assessed by 2 self-assessment questionnaires, including the 5-item version of the International Index of Erectile Function (IIEF-5) and the modified Brief Sexual Symptom Checklist (mBSSC). Median time between HSCT and genital examination was 5.9 years (range, 1 to 30.3 years). Thirty-one of 155 patients (20%) presented with genital skin changes. Twenty-one of those (13%) presented clinically inflammatory genital skin changes considered as genital cGVHD: 12 had inflammatory (noninfectious) balanoposthitis, 6 had lichen sclerosis-like lesions, 5 had phimosis, and 2 patients had more than 1 feature. Patients with inflammatory genital skin changes had a significantly higher coincidence of oral (P < .0001), ocular (P < .002), and/or cutaneous cGVHD (P < .026) when compared with patients without genital lesions. The rate of IIEF-5 questionnaire response was 59% (91 of 155). Among them, 67% reported erectile dysfunction. Erectile dysfunction was significantly more frequent in patients with genital cGVHD (P = .0075). Seventy-five of 155 patients (48%) answered the mBSSC questionnaire. Only 40% of them reported sexual contentment. Genital skin changes in male recipients after allogeneic HSCT are frequent and seem to be an under-reported relevant late effect. Inflammatory genital skin changes can be considered as a form of genital cGVHD often associated with manifestations of extragenital mucocutaneous cGVHD.

Pityriasis rubra pilaris: treatment with biologics - a new promising therapy?

Pityriasis rubra pilaris (PRP) includes a spectrum of rare chronic inflammatory disorders with papulosquamous eruptions of unknown cause. Different etiologies have been proposed such as vitamin A metabolism dysfunction, association with autoimmune disorders, infection or malignancies. However, PRP seems to be a polygenic skin disorder. Classical systemic treatment is empirical and includes retinoids and methotrexate; however, only few series on treatments exist. Recently there has been an increasing number of reports documenting that new biologicals and in particular TNF-α blockers are safe and effective.

Muckle-Wells syndrome effectively treated with canakinumab: is the recommended dosing schedule mandatory?

Cryopyrin-associated periodic fever syndrome (CAPS) is a rare inherited disease due to mutations in the NLRP3 (also called CIAS1) gene on chromosome 1q44 resulting in overproduction of interleukin-1. CAPS comprises three clinically overlapping disorders including Muckle-Wells syndrome. We report on two half-siblings with Muckle-Wells syndrome who were successfully treated with the interleukin-1 beta antibody canakinumab. Despite reduced dosing and longer treatment intervals compared to the recommended dosing schedule (e.g. 150 mg every 8 weeks), the efficacy and tolerability of canakinumab was impressive in both patients. The pharmacologic properties of canakinumab are reviewed and the clinical and economical aspects highlighted. We show that with individualized 'reflare-guided' administrations of canakinumab overall costs could hypothetically be reduced by 50% (approx. USD 60,000/patient/year) and therefore could have a major impact on treatment costs.

Buschke-Ollendorff syndrome in a three-generation family: influence of a novel LEMD3 mutation to tropoelastin expression.

Buschke-Ollendorff syndrome refers to the concomitant occurrence of connective tissue nevi, composed of elastic fibers in most cases, with osteopoikilosis. This autosomal dominant inherited disorder is caused by mutations in the gene LEMD3 on chromosome 12q14, which induces a rather heterogeneous clinical phenotype. Here, we report on the most proximal germline mutation found to date in the LEMD3 gene, p.Val94fs, in a three-generation Swiss family. Quantitative RNA analyses in affected and non-affected skin tissue from the proband demonstrate a comparable nonsense-mediated decay of mutant LEMD3 mRNA in both tissues; however, different levels of tropoelastin expression suggest that additional factors are involved in the development of the cutaneous lesions.