[HANSEN'S DISEASE IN ISRAEL].

INTRODUCTION: Hansen's disease (HD) is an anthropophylic, infectious, chronic disease, caused by Mycobacterium leprae. The systemic disease, affecting both males and females at any age, involves mainly the skin and the peripheral nerves located in the limbs close to the skin. Traditionally the treatment of HD patients was entrusted to dermatologists. Current drug therapy given at an early stage can prevent many of the complications, and enables patients to go on with life. In 95% of the population there is an innate immunity, which following exposure, enables the development of an effective immune response preventing the development of overt clinical disease. So far, there is neither an effective vaccination nor a simple test which can predict susceptibility to the infection. A long history of ignorance and stigmatization may add a social dimension to the physical ailment and suffering of HD patients. Despite the elimination campaign declared by the WHO in the early 1990s, the disease continues to exist. New patients are diagnosed each year, though in lower numbers and HD patients can be found all over the world. The relatively low prevalence of HD in non-endemic countries and the misconception that it has been eradicated long ago, make the awareness to the disease extremely poor. HD is usually not included in the differential diagnosis even when clear symptoms are present. This results in a very late diagnosis of new HD patients, thus missing the window of opportunity for early treatment which could prevent complications and disability. The purpose of this update is to raise awareness to the existence of HD in Israel and its diagnosis, to present updated epidemiological data for Israel and a glimpse at the global situation.

Insulin-like growth factor-binding protein-7 (IGFBP7) transcript: A-to-I editing events in normal and cancerous human keratinocytes.

Non-melanoma skin cancers (NMSC) are the most common malignancies in caucasians worldwide. Insulin-like growth factor-binding protein-7 (IGFBP7) was suggested to function as a tumor suppressor gene in several cancers, and to play a role in the proliferation of keratinocytes. A-to-I RNA editing is a post-transcriptional mechanism frequently used to expand and diversify transcriptome and proteome repertoire in eukaryotic cells. A-to-I RNA editing can alter codons, substitute amino acids and affect protein sequence, structure, and function. Two editing sites were identified within the IGFBP7 transcript. To evaluate the expression and editing of IGFBP7 mRNA in NMSC compared to normal epidermis. We examined the expression and mRNA editing level of IGFBP7 in 22 basal cell carcinoma (BCC), 15 squamous cell carcinoma (SCC), and 18 normal epidermis samples that were surgically removed from patients by the Mohs Micrographic Surgery procedure. We studied the effect of IGFBP7 editing on an immortalized HaCaT keratinocyte cell model. IGFBP7 mRNA is over expressed in BCC and SCC compared to normal epidermis. Moreover, the IGFBP7 transcript is highly edited in normal epidermis, but its editing is significantly reduced in BCC and SCC. The edited form of IGFBP7 can inhibit proliferation and induce senescence in cultured keratinocytes. This study describes for the first time A-to-I editing in the coding sequence of a tumor suppressor gene in humans, and suggests that IGFBP7 editing serves as a fine-tuning mechanism to maintain the equilibrium between proliferation and senescence in normal skin.

[Physicians' knowledge in Israel on the biology and control of head lice].

BACKGROUND: Health providers such as physicians, nurses and pharmacists should be knowledgeable about the biology of head lice and the ways to control them effectively, in order to reduce the proportion of children infested with head lice. OBJECTIVES: To evaluate the knowledge of physicians in Israel on the biology and epidemiology of lice, as well as their experience with infested individuals and their preferences for diagnosis, prophylaxis and control. METHODS: An anonymous questionnaire with 37 questions was used. The first 20 questions addressed the general knowledge of physicians on lice biology and control, while the remaining 17 questions were related to their personal experience with lice and louse treatment. RESULTS: Out of 273 physicians interviewed 66.8% had good knowledge of lice, while the remaining 33.2% had some knowledge on lice. The difference between the groups of physicians with medium and good knowledge on lice was borderline significant (P=0.0722), with the dermatologists borderline significantly less knowledgeable than the rest (P=0.0765). Significant differences were found between those physicians with 4-6 or 11-20 years of professional experience and the remaining groups (twice P<0.001). Although the percentage of female physicians who had a good knowledge on louse biology and control was higher than male physicians (39.4% and 29.4%, respectively), the differences were borderline significant (P=0.09). Pediatricians and dermatologists examined significantly more children than family physicians and general practitioners (P <0.001). CONCLUSIONS: The results of this study suggest that healthcare professionals' knowledge is of paramount importance for the correct diagnosis and control of head louse infestations.

The use of hybrid radiofrequency device for the treatment of rhytides and lax skin.

BACKGROUND: Recently, radiofrequency (RF) devices have been introduced and commercialized for nonablative procedures in dermatology and plastic surgery for the treatment of age-related rhytides and lax skin. OBJECTIVE: The objective was to assess the efficacy and safety of a novel RF device (Accent, Alma Lasers, Ltd, Caesarea, Israel) for the treatment of rhytides and lax skin. METHODS AND MATERIALS: Sixteen female patients (age range, 29-66 years; mean, 47+/-6 years; skin phototypes II to IV) were treated with Accent system. Patients were treated on the chin (n=5), forehead (n=8), cheeks (n=12), jowl lines (n=9), periorbital area (n=7), marionette line (n=3), and nasolabial folds (n=6) for wrinkles (n=27 cases) and skin laxity (n=23 cases). Patients received two to six treatments (mean, 4.3+/-1.1), with the time interval of 2 to 3 weeks. Photographs were assessed 1 month after the last treatment. RESULTS For wrinkles and skin laxity, in 5 patients (42%), the cheeks (n=12) scored 51% to 75% (significant improvement), and 2 patients (17%), 76% to 100% (excellent improvement). For the jowl lines (n=9), 4 patients (44%) scored 51% to 75% (significant improvement), and 1 patient scored 76% to 100% (excellent improvement) for lax skin. For wrinkles on the periorbital (n=7) and forehead areas (n=8), three patients (37%) scored 51% to 75% (significant improvement). CONCLUSION: The Accent system is an effective and safe modality for the improvement of age-related rhytides and lax skin.

Fatal hemophagocytic syndrome in a patient with panniculitis-like T-cell lymphoma and no clinical evidence of disease.

Panniculitis-like T-cell lymphoma is an uncommon type of extranodal T-cell lymphoma which presents clinically with subcutaneous nodules. The clinical course can either be indolent or rapidly progressive, often complicated by hemophagocytic syndrome. We report a patient with primary subcutaneous disease and initial complete response to combination chemotherapy. The patient experienced an early relapse which responded to salvage chemotherapy. However, she died shortly thereafter with hemophagocytic syndrome, polymicrobial sepsis and systemic fungal infection. At autopsy there was no evidence of lymphoma in the bone marrow or other organs. We emphasize that a fatal hemophagocytic syndrome can occur despite minimal or even without evidence: of clinically active lymphoma as demonstrated by autopsy in this case.

Diagnostic performance and retention of acquired skills after dermatology elective.

BACKGROUND: The dermatology elective often constitutes the future physician's only exposure to dermatologic practice. Cost-benefit considerations dictate that the elective enables the students to acquire useful diagnostic expertise in the short time period available, and that this expertise is not rapidly forgotten after completion of the elective. METHODS: In order to determine the efficacy of a 2-week dermatology elective in promoting diagnostic skills and to ascertain the preservation of the acquired expertise, kodachrome slide-based multiple-choice examinations were administered to 84 sixth year medical students at the beginning (pre-test) and completion (post-test) of the elective. The long-term retention of diagnostic skills was evaluated by re-testing 4-9 months after completion of the elective (follow-up test). RESULTS: The mean scores of correct answers at the pre- and post-test were 39.73% and 72.46%, respectively. The mean score at the follow-up test for students with a previous dermatology elective was 80.22%, compared with a score of 46.00% for students who did not participate in the elective. CONCLUSIONS: The 2-week dermatology elective significantly improved the diagnostic skills of the participants, and the knowledge obtained during the elective was retained for at least 4-9 months.

A nonsense mutation in the desmoglein 1 gene underlies striate keratoderma.

Striate keratodermas (PPKS) (OMIM 148700) are a rare group of autosomal dominant genodermatoses characterized by palmoplantar keratoderma typified by streaking hyperkeratosis along each finger and extending onto the palm of the hand. We report a four-generation kindred originating from Iran-Syria in which three members were affected with PPKS. Clinically, these patients present with hyperkeratotic palms and plantar plaques. Direct DNA sequencing analysis revealed a heterozygous C-to-A transversion at nt 395 of the DSG1 gene. This mutation converted a serine residue (TCA) in exon 5 to a nonsense mutation (TAA) designated S132X. The mutation identified in this study is a novel mutation in the DSG1 gene and extends the body of evidence implicating the desmoglein gene family in the pathogenesis of human skin disorders.

[Treatment of cutaneous leishmaniasis--an update].

Cutaneous leishmaniasis (CL) is a skin disease that manifests as ulcerated nodules, up to several centimeters in size, that persist for many months in spite of various therapies. The disease is a dermal infection with eukaryotic protozoa of the genus Leishmania, transferred by the bite of a female sand-fly, and therefore usually present on exposed areas of the skin such as limbs and face. The disease is endemic in specific geographical areas of Israel, mainly in the Jordan and Jericho valley, part of the Dead-Sea area, Kfar Edomim area, and parts of the Negev such as Ketziot and the Arava. Cutaneous leishmaniasis (CL) presents a significant health hazard in Israel, mainly to non-residents, such as soldiers or new immigrants, travelling to endemic areas. It is also not unusual to find patients with CL acquired during adventure travelling to South America. Treatment modalities available for treating CL are limited and often unpleasant. Over the past few years several new therapies for visceral leishmaniasis have become available raising hope for the development of more effective treatment options for CL. In this review we discuss the disease and its therapeutic options and also report a novel treatment for CL using ethanolic amphotericin B.