Publications by authors named "Michael Ziv"

47 Publications

Interviewing Women with Hidradenitis Suppurativa-Thematic and Content Analysis.

Adv Skin Wound Care 2022 Jul;35(7):381-384

Shani Fisher, MA, RN, is Chief Nurse in the Dermatology and Venereology Department, Emek Medical Center, Afula, Israel, and the Department of Nursing, Steyer School of Health Professions, Sackler School of Medicine, Tel Aviv University, Israel. Michael Ziv, MD, is Head of Department, Dermatology and Venereology Department, Emek Medical Center. The authors have disclosed no financial relationships related to this article. Submitted July 21, 2021; accepted in revised form August 11, 2021.

Objective: Hidradenitis suppurativa (HS) is a chronic autoinflammatory skin disease with a higher prevalence in women. The disease results in a low quality of life as well as physical and psychological comorbidities. The authors sought to determine the effects of HS on women's self-perception and life experiences.

Methods: Semistructured interviews were conducted with 22 women of varying age and family status. The content was transcribed and subjected to both thematic and content analyses.

Results: Five themes and a number of subthemes were revealed, involving physical, emotional, coping, and functional aspects. Somatic features, especially pain, were the most troubling issues, along with the emotional burden of shame and loss of femininity and intimacy. However, women also revealed strength and expressed optimism.

Conclusions: These findings reveal the inner world of women coping with HS, addressing multiple dilemmas, problems, and concerns. Healthcare providers should pay special attention to the specific needs of these patients. Additional research is needed to further shed light on the impact of HS on women.
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http://dx.doi.org/10.1097/01.ASW.0000831084.75243.66DOI Listing
July 2022

Phototherapy for Generalized Pruritus of Unknown Origin: Single-Center Experience.

Adv Skin Wound Care 2022 Feb;35(2):109-111

In the Dermatology and Venereology Department, Emek Medical Center, Afula, Israel, Shani Fisher, MA, RN, is Chief Nurse, and Michael Ziv, MD, is Department Head. The authors have disclosed no financial relationships related to this article. Submitted March 17, 2021; accepted in revised form April 21, 2021.

Objective: Phototherapy is a well-established therapy in dermatology. However, there is limited evidence regarding phototherapy for the treatment of generalized pruritus of unknown origin (GPUO). The objective of this study was to assess the efficacy and safety of narrowband ultraviolet B (NB-UVB) phototherapy in patients with GPUO.

Methods: Researchers conducted a retrospective review of the treatment outcomes of patients with GPUO who were treated with NB-UVB between 2004 and 2019 at their facility.

Results: Investigators included 67 patients diagnosed with GPUO treated with NB-UVB. Complete remission was achieved in more than 70% of the patients. No serious adverse events were documented.

Conclusions: For patients with GPUO, NB-UVB may be a safe and effective treatment option.
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http://dx.doi.org/10.1097/01.ASW.0000803256.62585.d5DOI Listing
February 2022

Concomitant variants in NF1, LZTR1 and GNAZ genes probably contribute to the aggressiveness of plexiform neurofibroma and warrant treatment with MEK inhibitor.

Exp Dermatol 2022 05 20;31(5):775-780. Epub 2021 Dec 20.

Bruce and Ruth Rappaport Faculty of Medicine, Technion, Haifa, Israel.

Neurofibromatosis 1 (NF1) is caused by germline mutations in the NF1 gene and manifests as proliferation of various tissues, including plexiform neurofibromas. The plexiform neurofibroma phenotype varies from indolent to locally aggressive, suggesting contributions of other modifiers in addition to somatic loss of NF1. In this study, we investigated a life-threatening plexiform neurofibroma in a 9-month-old female infant with NF1. Germline mutations in two RASopathy-associated genes were identified using whole-exome sequencing-a de novo pathogenic variant in the NF1 gene, and a known pathogenic variant in the LZTR1 gene. Somatic analysis of the plexiform neurofibroma revealed NF1 loss of heterozygosity and a variant in GNAZ, a gene encoding a G protein-coupled receptor. Cells expressing mutant GNAZ exhibited increased ERK 1/2 activation compared to those expressing wild-type GNAZ. Taken together, we suggest the variants in NF1, LZRT1 and GNAZ act synergistically in our patient, leading to MAPK pathway activation and contributing to the severity of the patient's plexiform neurofibromatosis. After treatment with the MEK inhibitor, trametinib, a prominent clinical improvement was observed in this patient. This case study contributes to the knowledge of germline and somatic non-NF1 variants affecting the NF1 clinical phenotype and supports use of personalized, targeted therapy.
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http://dx.doi.org/10.1111/exd.14514DOI Listing
May 2022

Parental mosaic cutaneous-gonadal GJB2 mutation: From epidermal nevus to inherited ichthyosis-deafness syndrome.

J Dermatol 2022 Mar 9;49(3):379-382. Epub 2021 Dec 9.

Department of Dermatology, "Emek" Medical Center, Afula, Israel.

Ichthyosis and deafness syndrome is a group of devastating genodermatoses caused by heterozygous mutations in GJB2, encoding the gap junction protein connexin 26. These syndromes are characterized by severe skin disease, hearing loss, recurrent infections, and cutaneous neoplasms. Cutaneous somatic mutations in the same gene are associated with porokeratotic eccrine ostial dermal duct nevus. Here we report a family in which a parent presented with localized epidermal nevus and his child suffered with hystrix-like ichthyosis with deafness. Histologic examination of the parent's cutaneous lesion revealed verrucous epidermal nevus without features of porokeratotic eccrine ostial dermal duct nevus. Genetic analysis identified the same pathogenic variant, GJB2 c.148G>A (p.D50N), in DNA extracted from the parent's cutaneous lesion and the child's leukocytes, but not in the parent's leukocytes. This study expands the phenotypic heterogeneity of GJB2 mosaic variants in addition to porokeratotic eccrine ostial dermal duct nevus, and emphasizes the importance of molecular diagnosis of mosaic skin diseases considering the risk of severe inherited diseases in the offspring.
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http://dx.doi.org/10.1111/1346-8138.16268DOI Listing
March 2022

Acral peeling in Nagashima type palmo-plantar keratosis patients reveals the role of serine protease inhibitor B 7 in keratinocyte adhesion.

Exp Dermatol 2022 02 17;31(2):214-222. Epub 2021 Aug 17.

Bruce and Ruth Rappaport Faculty of Medicine, Technion, Haifa, Israel.

Acral peeling skin syndrome (APSS) is a heterogenous group of genodermatoses, manifested by peeling of palmo-plantar skin and occasionally associated with erythema and epidermal thickening. A subset of APSS is caused by mutations in protease inhibitor encoding genes, resulting in unopposed protease activity and desmosomal degradation and/or mis-localization, leading to enhanced epidermal desquamation. We investigated two Arab-Muslim siblings with mild keratoderma and prominent APSS since infancy. Genetic analysis disclosed a homozygous mutation in SERPINB7, c.796C > T, which is the founder mutation in Nagashima type palmo-plantar keratosis (NPPK). Although not previously formally reported, APSS was found in other patients with NPPK. We hypothesized that loss of SERPINB7 function might contribute to the peeling phenotype through impairment of keratinocyte adhesion, similar to other protease inhibitor mutations that cause APSS. Mis-localization of desmosomal components was observed in a patient plantar biopsy compared with a biopsy from an age- and gender-matched healthy control. Silencing of SERPINB7 in normal human epidermal keratinocytes led to increased cell sheet fragmentation upon mechanical stress. Immunostaining showed reduced expression of desmoglein 1 and desmocollin 1. This study shows that in addition to stratum corneum perturbation, loss of SERPINB7 disrupts desmosomal components, which could lead to desquamation, manifested by skin peeling.
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http://dx.doi.org/10.1111/exd.14444DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8831670PMC
February 2022

Skin and soft tissue infections in biological therapy for psoriasis-A case report and systematic review of the literature.

Int J Dermatol 2021 Nov 3;60(11):1429-1434. Epub 2021 Jun 3.

Dermatology and Venereology Department, Emek Medical Center, Afula, Israel.

Background: Biological therapies are widely used for moderate to severe chronic plaque psoriasis owing to their high efficacy and safety profile. However, skin and soft tissue infections (SSTIs) have been reported in association with biological treatment in psoriasis.

Methods: We report a case of necrotizing fasciitis in an 18-year-old psoriasis patient with a history of severe combined immunodeficiency treated with secukinumab and conducted a systematic literature review of SSTIs associated with biological therapy for psoriasis. The literature review related to biological therapies for psoriasis between the years 1990 and 2020: Medline (PubMed), Embase, and CENTRAL (Cochrane Central Register of Controlled Trials) were searched for psoriasis, biological treatment, and skin and soft tissue infections.

Results: Over 1,300 titles were found, 24 of which met the inclusion criteria for our study: nine retrospective studies, nine randomized controlled trials, and six prospective studies. The data covered 10 biological treatments. More than 40,000 patients receiving biological treatment were included, and nearly 1,000 cases of SSTIs were documented.

Conclusions: We present the available records regarding SSTIs among chronic plaque psoriasis patients given biological treatment. Most reported SSTIs were related to psoriasis patients treated with TNF-α inhibitors. In view of the presented data, biological treatment appears to be a safe mode of therapy for this aspect of psoriasis.
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http://dx.doi.org/10.1111/ijd.15679DOI Listing
November 2021

LEPOARD syndrome: A report of a case with a novel PTPN11 mutation.

JAAD Case Rep 2021 May 20;11:57-59. Epub 2021 Mar 20.

Department of Dermatology, Ha'emek Medical Center, Afula, Israel.

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http://dx.doi.org/10.1016/j.jdcr.2021.03.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8054096PMC
May 2021

Ophthalmic Assessment in Patients With Darier Disease.

Am J Ophthalmol 2021 07 15;227:139-142. Epub 2021 Mar 15.

From the Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel (H.H., E.A., A.Y., D.B., M.Z., R.P.D.-G.); Department of Dermatology, Emek Medical Center, Afula, Israel (H.H., A.Y., M.Z., R.P.D.-G.); Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada (R.P.D.-G.).. Electronic address:

Purpose: To assess the prevalence of ophthalmic findings in patients with Darier disease, an autosomal dominant genetic skin disorder, in an effort to evaluate the need for eye examinations in the management of the disease.

Design: Prospective observational case series.

Methods: Thirty-six individuals with Darier disease were evaluated by both ocular assessment questionnaire and a comprehensive ophthalmic examination (visual acuity, refraction, external examination, and slit-lamp examination) with emphasis on the eyelids, conjunctiva, and cornea. In addition, questionnaire-based medical interview and skin examination were conducted.

Results: According to the medical questionnaire, 39% of patients reported eye problems, 36% dry eye, and 42% eye fatigue after prolonged reading. Ocular examination revealed Darier disease lesions on the eyelids in 55% of the patients, blepharitis in 44%, conjunctival hyperemia in 28%, and short tear film break-up time in 83%. There was no significant relationship between any of these ophthalmic findings and systemic retinoid therapy, sex, or age.

Conclusions: The high prevalence of blepharitis and dry eye highlights the importance of ophthalmologic evaluation of patients with Darier disease.
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http://dx.doi.org/10.1016/j.ajo.2021.03.011DOI Listing
July 2021

An Update on the Cutaneous Manifestations of Darier Disease.

J Cutan Med Surg 2021 Sep 9;25(5):498-503. Epub 2021 Mar 9.

26747 Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.

Background: Knowledge about the clinical features of Darier disease, an orphan autosomal-dominant genetic disorder, is sparse and has been evaluated only in few studies.

Objectives: To investigate the clinical features of a large group of patients with Darier disease, and to explore for associations between disease characteristics and severity of the disease.

Methods: Seventy-six individuals with Darier disease were evaluated utilizing a structured questionnaire-based interview, a physical examination, and a retrospective assessment of their medical records.

Results: The most frequent locations of lesions were hands (99%) and fingernails (93%). Wart-like lesions on the hands were more visible after soaking them in water for 5 minutes, we therefore named this phenomenon the "wet hand sign". Oral involvement was found in 43% of patients, while 48% of women and 16% of men showed genital lesions. Patients with severe Darier disease had a tenfold greater risk of developing genital lesions than those with mild disease ( = .01). Most patients (88%) in our study exhibited a combination of the four types of the disease patterns of distribution (flexural, seborrheic, nevoid, and acral).

Conclusions: Documentation of disease on the hands and fingernails provides a highly sensitive means to aid in the diagnosis of Darier disease. It is important to evaluate mucosal lesions including genital and oral mucosa.
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http://dx.doi.org/10.1177/1203475421999331DOI Listing
September 2021

Retrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment.

J Cutan Med Surg 2021 May-Jun;25(3):271-280. Epub 2021 Jan 4.

494622 Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are adverse drug reactions.

Objectives: To learn about the clinical characteristics of patients with SJS/TEN including treatments provided, outcomes, and mortality.

Methods: We conducted a retrospective chart review of patients who were hospitalized with the diagnosis of SJS/TEN at the Ross Tilley Burn Center between the years 1999 and 2015.

Results: A total of 43 patients were identified with a mean age of 54 ± 19 (58, 18-85). The most common offending medications were allopurinol and carbamazepine. The overall mortality rate in our study is 21% with the most common causes of death being multiorgan failure and sepsis. The majority of our patients had oral (84%), ocular (79%), and genital (60%) involvement during hospitalization. Our data revealed that combination treatment involving oral corticosteroids with intravenous immunoglobulin (IVIG) had the highest mortality rate in our study since 55% (6/11) of patients who were treated in this manner passed away compared to 11% (2/18) of patients passing away who were treated with solely IVIG and 33% (1/3) who were treated with only supportive care. Our study also demonstrates the addition of etanercept and cyclosporine treatment in the second time period we studied: 2008-2015 versus the earlier time period of 1999-2007. None of the patients in our study who were treated with therapies including cyclosporine and/or etanercept passed away.

Conclusions: Our study sheds light on a possible beneficial role of cyclosporine and etanercept for the treatment of SJS and TEN and reinforces the necessity of a multidisciplinary care team for patients.
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http://dx.doi.org/10.1177/1203475420982550DOI Listing
November 2021

Granulomatous Cutaneous Drug Eruptions: A Systematic Review.

Am J Clin Dermatol 2021 Jan;22(1):39-53

Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, ON, Canada.

Background: Granulomatous drug eruptions are rare entities, where granuloma formation occurs as an attempt to contain an exogenous or endogenous inciting agent. Granulomatous drug eruptions may be localized to the skin or may include major systemic involvement, and their characteristics depend both on the properties of the causative irritant and host factors. Because of the overlapping features amongst noninfectious granulomatous diseases, granulomatous drug eruptions are challenging to diagnose and distinguish both histologically and clinically.

Objective: The objective of this article is to provide a review and summary of the current literature on the five major types of cutaneous granulomatous drug eruptions: interstitial granulomatous drug reaction, drug-induced accelerated rheumatoid nodulosis, drug-induced granuloma annulare, drug-induced sarcoidosis, and miscellaneous presentations.

Methods: A systematic review was conducted through PubMed using the search terms "granulomatous drug eruption" and "cutaneous" or "skin". English full-text studies that included human subjects experiencing a cutaneous reaction comprising granulomatous inflammation as the direct result of a drug were included. Of 205 studies identified, 48 articles were selected after a full-text review. Evidence was evaluated using the Tool for evaluating the methodological quality of case reports and case series.

Results: Polypharmacy and a prolonged lag period from drug ingestion to rash onset may create diagnostic challenges. Ruling out tuberculosis is imperative in the endemic setting, particularly where anti-tumor necrosis factor therapy is the presumed cause. Interstitial granulomatous drug reactions and granuloma annulare are often localized to the skin whereas accelerated rheumatoid nodulosis and sarcoidosis may sometimes be associated with systemic features as well. Granulomatous drug eruptions typically resolve on discontinuing the offending medication; however, the decision for drug cessation is dependent on a risk-benefit assessment. In some situations, supplementation of an additional agent to suppress the reaction may resolve symptoms. In some cases, granulomatous drug eruptions may be pivotal in the successful outcome of the drug, as in cases of melanoma treatment. In all situations, the decision to continue or withdraw the drug should be carefully based on the severity of the eruption, necessity of continuing the drug, and availability of a suitable alternative.

Conclusions: Granulomatous drug eruptions should always be considered in the differential diagnosis of noninfectious granulomatous diseases of the skin. Further research examining dose-response relationships and the recurrence of granulomatous drug eruptions on the rechallenge of offending agents is required. Increased awareness of granulomatous drug eruption types is important, especially with continuous development of new anti-cancer agents that may induce these reactions.

Clinical Trial Registration: PROSPERO registration number CRD42020157009.
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http://dx.doi.org/10.1007/s40257-020-00566-4DOI Listing
January 2021

[PIGMENTED BASAL CELL CARCINOMA AND IONIZING RADIATION].

Harefuah 2020 Aug;159(8):541-544

Department of Plastic Surgery, "Emek" Medical Center, Afula, Israel.

Aims: This study aimed at demonstrating whether the histological and clinical manifestations of pigmented basal cell carcinoma are different among those who were previously treated with ionizing radiation for ringworm infection.

Background: Ionizing radiation is known to cause increased morbidity among those who are chronically exposed. Basal cell carcinoma in known to be related to ionizing radiation however, the characteristics of pigmented BCC in relation to ionizing radiation are poorly described.

Methods: The study included the demographics and characteristics of 23 patients with pigmented BCC who were treated for ringworm with ionization radiation and a control group of 21 patients that had not been treated with ionizing radiation. All the cases treated between the years 2005-2015 were included in the study. The data was analyzed with a SPSS program.

Results: Among the patients who were treated with ionizing radiation the percentage of the tumors that were well differentiated was 34.8%, much higher than those who were not treated with ionizing radiation - 14.3%. In addition, the average age for those who were treated with ionizing radiation was 66 compared to 73 in the group that weren't treated with radiation.

Discussion: Pigmented basal cell carcinoma is a rare variant of BCC and it has characteristics that are quite dissimilar among patients treated with ionizing radiation. However, more studies are needed in order to strengthen the results.
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August 2020

Infliximab-induced neutrophilic eccrine hidradenitis in a patient with hidradenitis suppurativa.

Dermatol Ther 2020 11 28;33(6):e13900. Epub 2020 Jul 28.

Department of Dermatology, Emek Medical Center Affiliated with the Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Afula, Israel.

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http://dx.doi.org/10.1111/dth.13900DOI Listing
November 2020

Efficacy of topical ichthammol 10% for hidradenitis suppurativa: Case series and systematic review of its use in dermatology.

Dermatol Ther 2020 11 6;33(6):e13868. Epub 2020 Jul 6.

Dermatology and Venereology Clinic, Emek Medical Center, Afula, Israel.

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http://dx.doi.org/10.1111/dth.13868DOI Listing
November 2020

Cutaneous manifestations of COVID-19: Report of three cases and a review of literature.

J Dermatol Sci 2020 May 29;98(2):75-81. Epub 2020 Apr 29.

Dermatology Department, Emek Medical Center, Israel; Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Israel; Department of Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address:

Background: Various cutaneous manifestations have been observed in patients with COVID-19 infection. However, overall similarities in the clinical presentation of these dermatological manifestations have not yet been summarized.

Objective: This review aims to provide an overview of various cutaneous manifestations in patients with COVID-19 through three case reports and a literature review.

Methods: A literature search was conducted using PubMed, OVID, and Google search engines for original and review articles. Studies written in the English language that mentioned cutaneous symptoms and COVID-19 were included.

Results: Eighteen articles and three additional cases reported in this paper were included in this review. Of these studies, 6 are case series and 12 are case report studies. The most common cutaneous manifestation of COVID-19 was found to be maculopapular exanthem (morbilliform), presenting in 36.1% (26/72) patients. The other cutaneous manifestations included: a papulovesicular rash (34.7%, 25/72), urticaria (9.7%, 7/72), painful acral red purple papules (15.3%, 11/72) of patients, livedo reticularis lesions (2.8%, 2/72) and petechiae (1.4%, 1/72). Majority of lesions were localized on the trunk (66.7%, 50/72), however, 19.4% (14/72) of patients experienced cutaneous manifestations in the hands and feet. Skin lesion development occurred before the onset of respiratory symptoms or COVID-19 diagnosis in 12.5% (9/72) of the patients, and lesions spontaneously healed in all patients within 10 days. Majority of the studies reported no correlation between COVID-19 severity and skin lesions.

Conclusion: Infection with COVID-19 may result in dermatological manifestations with various clinical presentations, which may aid in the timely diagnosis of this infection.
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http://dx.doi.org/10.1016/j.jdermsci.2020.04.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189855PMC
May 2020

Risks of hydroxychloroquine use for COVID-19 prophylaxis.

J Am Acad Dermatol 2020 07 26;83(1):e73-e74. Epub 2020 Apr 26.

Dermatology Department, Emek Medical Center, Afula, Israel; School of Medicine, Tel Aviv University, Tel Aviv, Israel; Bruce Rappaport Faculty of Medicine, Technion-Institute of Technology, Haifa, Israel; Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1016/j.jaad.2020.04.111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195137PMC
July 2020

Vemurafenib-induced DRESS/DIHS resulting in spontaneous melanoma regression: an immunological reaction shedding new light on melanoma treatment?

Int J Dermatol 2020 May 25;59(5):e139-e141. Epub 2020 Mar 25.

Ella Lemelbaum Institute for Immuno-Oncology, Sheba Medical Center, Ramat-Gan, Israel.

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http://dx.doi.org/10.1111/ijd.14852DOI Listing
May 2020

[SYSTEMIC REVIEW OF EOSINOPHILIC DERMATOSES PATIENTS TREATED WITH TNF-Α INHIBITORS AND USTEKINUMAB].

Harefuah 2020 Jan;159(1):34-37

Department of Dermatology, Emek Medical Center, Afula, Israel.

Aims: This study aims to critically review the pros and cons of biological drugs as treatments and triggers of eosinophilic dermatoses.

Background: Eosinophilic dermatoses syndromes are rare diseases with a prominent eosinophilic infiltration mechanism. These syndromes have several known treatments with limited success. Several physicians worldwide suggested possible advantages of using specific biological drugs, which are different from eosinophil targeted biotherapies as treatments for eosinophilic dermatoses syndromes. Others considered these drugs as possible triggers.

Methods: Articles published in the last 30 years containing relevant key words were reviewed using PubMed and Medline. Associations between Infliximab, Adalimumab, Etanercept, TNF alpha inhibitors and Ustekinumab to Eosinophilic Dermatoses syndromes were reviewed.

Results: Our search revealed an association between 17 eosinophilic dermatoses patients and the drugs of interest. Out of 5 Wells' syndrome cases, four patients had an outbreak of the disease following treatment and one improved by the treatment. Six cases of Eosinophilic Fasciitis mostly had a positive reaction to the treatment. More associations were found among 4 cases of Churg-Strauss syndrome, one case of Granuloma Faciale and 1 case of Eosinophilic Pustular Folliculitis.

Conclusions: TNF alpha inhibitors and Ustekinumab may have a role in the treatment of eosinophilic dermatosis syndromes. These drugs may act as triggers among Wells' syndrome patients. Further investigation is needed.
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January 2020

The Role of Desmoglein 1 in Gap Junction Turnover Revealed through the Study of SAM Syndrome.

J Invest Dermatol 2020 03 26;140(3):556-567.e9. Epub 2019 Aug 26.

Departments of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA; Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA; Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USA. Electronic address:

An effective epidermal barrier requires structural and functional integration of adherens junctions, tight junctions, gap junctions (GJ), and desmosomes. Desmosomes govern epidermal integrity while GJs facilitate small molecule transfer across cell membranes. Some patients with severe dermatitis, multiple allergies, and metabolic wasting (SAM) syndrome, caused by biallelic desmoglein 1 (DSG1) mutations, exhibit skin lesions reminiscent of erythrokeratodermia variabilis, caused by mutations in connexin (Cx) genes. We, therefore, examined whether SAM syndrome-causing DSG1 mutations interfere with Cx expression and GJ function. Lesional skin biopsies from SAM syndrome patients (n = 7) revealed decreased Dsg1 and Cx43 plasma membrane localization compared with control and nonlesional skin. Cultured keratinocytes and organotypic skin equivalents depleted of Dsg1 exhibited reduced Cx43 expression, rescued upon re-introduction of wild-type Dsg1, but not Dsg1 constructs modeling SAM syndrome-causing mutations. Ectopic Dsg1 expression increased cell-cell dye transfer, which Cx43 silencing inhibited, suggesting that Dsg1 promotes GJ function through Cx43. As GJA1 gene expression was not decreased upon Dsg1 loss, we hypothesized that Cx43 reduction was due to enhanced protein degradation. Supporting this, PKC-dependent Cx43 S368 phosphorylation, which signals Cx43 turnover, increased after Dsg1 depletion, while lysosomal inhibition restored Cx43 levels. These data reveal a role for Dsg1 in regulating epidermal Cx43 turnover.
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http://dx.doi.org/10.1016/j.jid.2019.08.433DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7039747PMC
March 2020

Knowledge and opinions among Canadian academic physicians regarding genetic screening to prevent severe cutaneous adverse drug reactions.

J Am Acad Dermatol 2019 12 19;81(6):1401-1405. Epub 2019 Apr 19.

Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Canada; The Ruth and Bruce Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa, Israel; Department of Dermatology, Emek Medical Centre, Afula, Israel. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2019.04.040DOI Listing
December 2019

Acetic acid treatment for toe web infection caused by Pseudomonas Aeruginosa combined with fungal infection: A case series of ten patients.

Dermatol Ther 2019 05 17;32(3):e12883. Epub 2019 Apr 17.

Department of Dermatology, "Emek" medical center, Afula, Israel.

Gram-negative bacterial toe web infection (GNBTWI) caused by Pseudomonas Aeruginosa combined with fungal infection has variety of treatments. However, these treatments have been poorly described in the literature. Our retrospective study describes patients that had been treated in our medical center with acetic acid combined with local antifungal treatment, to evaluate evidences for the appropriateness of this treatment. Ten patients with evidence of GNBTWI caused by Pseudomonas Aeruginosa combined with local fungal infection which have been treated with acetic acid in Emek Medical Center were identified. Eight patients (80%) had a complete response while two patients (20%) showed only a partial response. Side effects were minimal and included temporary stinging sensation. Acetic acid is a relatively cheap ingredient with minimal side effect profile and highly effective outcomes as a treatment for GNBTWI caused by Pseudomonas Aeruginosa and should be considered as an adjuvant treatment.
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http://dx.doi.org/10.1111/dth.12883DOI Listing
May 2019

Wells' Syndrome Induced by Ustekinumab.

Isr Med Assoc J 2019 Jan;21(1):65

Department of Dermatology, Emek Medical Center, Afula, Israel.

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January 2019

Mortality and risk factors among Israeli bullous pemphigoid patients.

Arch Dermatol Res 2019 Jan 31;311(1):19-27. Epub 2018 Oct 31.

Department of Dermatology, Emek Medical Center, Afula, Israel.

There are differences concerning reported mortality rates and prognostic factors of bullous pemphigoid (BP) patients in different studies. Our objectives were to evaluate the mortality rates and prognostic factors among Israeli BP patients compared to matched control subjects. Three age- and sex-matched patients without BP (n = 261) who were treated in our clinic were selected and compared to BP patients (n = 87). Mean survival period of the BP group was 4.1 years (95% CI: 3.3-4.8 years) and 5.9 years among the non-BP group (95% CI: 5.6-6.3 years). The 1-year mortality rate was 24.1% for the BP group and 6.5% for the control group. In multivariate analysis, age above 80 was a significant risk factor for mortality [HR 3.22 (95% CI, 1.15-8.96), p = 0.03], while statins intake had a protective role [HR 0.36 (95% CI, 0.15-0.88), p = 0.03]. In univariant analysis, dementia [HR 2.44 (95% CI, 1.02-5.99), p = 0.04] was a risk factor. In conclusion, BP patients' mortality is correlated to increasing age at diagnosis, dementia, and statins use. Statins' protective role is newly discussed in the literature.
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http://dx.doi.org/10.1007/s00403-018-1875-zDOI Listing
January 2019

Severe Physical Complications among Survivors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Drug Saf 2018 03;41(3):277-284

Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, 2075 Bayview Avenue, Suite MI-700, Toronto, ON, M4N 3M5, Canada.

Introduction: Few studies have reported the physical complications among Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) survivors.

Objective: The aim of this study was to comprehensively characterize the physical complications among SJS/TEN survivors and to learn about patients' perspectives of surviving SJS/TEN.

Methods: SJS/TEN survivors older than 18 years of age were assessed by different methods: a medical interview; a questionnaire assessing patients' perspectives; thorough skin, oral mucous membrane, and ophthalmic examinations; and a retrospective assessment of medical records.

Results: Our cohort consisted of 17 patients with a mean time of 51.6 ± 74.7 months (median 9, range 1-228) following SJS/TEN. The most common physical complications identified in the medical examination were post-inflammatory skin changes (77%), cutaneous scars (46%), dry eyes (44%), symblepharon, and chronic ocular surface inflammation (33% each). Novel physical sequelae included chronic fatigue (76%) and pruritus (53%). We also found a novel association between the number of mucous membranes affected in the acute phase of SJS/TEN and hair loss during the 6 months following hospital discharge; hair loss was reported in 88% of the group of patients who had three or more mucous membranes affected versus 29% of patients who had less than three mucous membranes involved (p = 0.0406). Following hospital discharge due to SJS/TEN, 59% of patients were followed by a dermatologist, although 88% had dermatological complications; 6% were followed by an ophthalmologist, even though 67% had ophthalmological complications; and 6% of female survivors were followed by a gynecologist, even though 27% had gynecological complications.

Conclusion: Survivors of SJS/TEN suffer from severe physical complications impacting their health and lives that are mostly under recognized and not sufficiently treated by medical professionals.
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http://dx.doi.org/10.1007/s40264-017-0608-0DOI Listing
March 2018

[CUTANEOUS T CELL LYMPHOMA: UNILESIONAL MYCOSIS FUNGOIDES].

Harefuah 2016 Oct;155(10):613-615

The Department of Dermatology, Haemek Medical Center, Afula, Israel.

Introduction: Unilesional mycosis fungoides is a rare cutaneous T cell lymphoma that warrants either radiation therapy or surgical excision. Benign characteristics result in misdiagnosis, delayed tissue biopsy and subsequently delayed provision of adequate treatment. A young patient presented with a history of 18 months of eczematous benign - appearing single lesion restricted to her index finger. Local electron-beam radiation following tissue diagnosis resulted in full recovery.
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October 2016
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