[Show abstract][Hide abstract] ABSTRACT: Background
Cutaneous leishmaniasis (CL) due to Leishmania major (L. major) is common in the Middle East; however, this skin infection may be under-diagnosed when it presents atypically.Objective
To highlight the occurrence of uncommon presentations of CL that may elude diagnosis.Materials and methodsA retrospective study was performed among patients who presented at The Sheba Medical Center between 2005 and 2014 with atypical clinical presentations of CL due to L. major.ResultsTwelve patients with unusual clinical presentations of L. major CL were identified. All infections were acquired in L. major – endemic areas of Israel. The average age was 37 years. The average number of lesions was 2. Nine patients presented with a form that mimicked other forms of CL, such as lupoid, giant ulcer, sporotrichoid and recidivans, and three had a variant resembling other infectious skin diseases, such as erysipeloid and verruciform. All patients required systemic therapy.Conclusion
Cutaneous leishmaniasis due to L. major can masquerade as many other infectious and inflammatory diseases. In addition, it can mimic clinical forms of New World CL. We suggest that in endemic countries or in travellers returning from countries where L. major is endemic, polymerase chain reaction (PCR) for Leishmania-specific DNA should be performed routinely in cases of unusual presentations of dermatitis with a single or a few lesions, even if a diagnosis of CL was not considered by the referring clinician.
Full-text · Article · Sep 2015 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: Abstract Background: Lichen planopilaris (LPP) is as a lymphocytic variant of primary cicatricial alopecia. Objective: To evaluate the demographics, clinical findings, natural history, and response to various treatments of LPP. Methods: A retrospective review of medical records of all patients with clinical and histopathological diagnosis of LPP. Results: Out of 46 patients there were 38 (82.6%) women and 8 (17.4%) men. There was no clear association of specific medical background and medications with disease onset. The most frequent complaint was itching scalp. Asymptomatic hair loss was observed in 39.2%. LPP involved the entire scalp in 39.1%, vertex in 28.3%, anterior scalp and vertex in 17.4%, fronto-temporal scalp in 6.5%, posterior and parietal scalp in 6.5%. The topical treatment that caused the highest rate of symptomatic improvement was intralesional injection of corticosteroids. The treatment that led to the highest rate of remission was hydroxychloroquine combined with topical corticosteroid application. The remission rate was 6.5% after 3 months, and 33% after 18 months. Of patients who achieved remission, 50% need continued treatment to maintain remission. No patient had any visible hair regrowth on any treatment. Conclusion: The range of empiric topical and systemic treatments used gives unsatisfactory results, in LPP patients.
No preview · Article · Jun 2014 · Journal of Dermatological Treatment
[Show abstract][Hide abstract] ABSTRACT: Background
Onchocerciasis is an infectious disease caused by the filaria Onchocerca volvulus. Very little is known regarding onchocerciasis imported from endemic to non-endemic areas.Objective
To evaluate pruritic dermatitis simulating atopic dermatitis in Ethiopian immigrants in Israel.Materials and MethodsA retrospective study on 27 Ethiopian immigrants to Israel was conducted. Demographics and clinical and laboratory data were collected.ResultsOf the group of 27 patients, ten (37%) were men and 17(63%) were women. The average age at referral was 29 years. All of the patients immigrated from Kuwara, Ethiopia. Diagnosis was done by either positive snip test or immunoglobulin IgG4 serology of onchocerciasis in 14 patients. The most common presentation was a combination of lichenified onchodermatitis with atrophy and depigmentation (36%). Eosinophilia and elevated IgE levels were common. Seventeen patients were treated with a single administration of oral ivermectin 200 mcg/mg. Thirteen patients responded to the treatment.Conclusions
Immigrants from endemic regions to developed countries presenting with pruritic diseases, especially those with a clinical picture suggestive of atopic dermatitis, should be and evaluated for possible onchocerciasis infection. Ivermectin, a relatively safe and low-cost treatment,should be considered even in the absence of a proven disease. Physicians should have a high index of suspicion in patients with the right residential history.This article is protected by copyright. All rights reserved.
No preview · Article · Mar 2014 · British Journal of Dermatology
[Show abstract][Hide abstract] ABSTRACT: Morphea and other scleroderma-like skin conditions are occasionally linked with exposure to chemical compounds such as silicone. We treated a 56-year-old woman with generalized severe skin induration accompanied with systemic symptoms and peripheral eosinophilia, which appeared 2.5 years after breast silicone implantation and abdominal liposuction. Blood test results and histopathological examination of her skin suggested the diagnosis of morphea overlapping with eosinophilic fasciitis. Her skin disease was presumed to be an autoimmune reaction to silicone implantation. While the removal of the implants did not improve her illness, treatment with 1 mg/kg prednisone and PUVA bath was initiated, with some improvement. This patient illustrates an example of ASIA (Autoimmune Syndrome Induced by Adjuvants), as her disease appeared following exposure to an adjuvant stimulus, with 'typical', although not well-defined, autoimmune manifestations.
[Show abstract][Hide abstract] ABSTRACT: Background 'Erysipelas-like' erythema (ELE) is a well recognized, although uncommon, manifestation of familial Mediterranean fever (FMF), which is frequently mistaken for infectious erysipelas, especially when forming the initial disease presentation. Aim To clinically and genetically characterize ELE as the first manifestation of FMF. Methods FMF patients with ELE as the first disease presentation (study group), were compared with FMF patients with ELE, appearing during the disease course (control group I), and to those FMF patients who never had ELE (control group II). Results Patients of the study group were comparable to patients without ELE with respect to all demographic, clinical and genetic features studied, and yet differed from patients with ELE appearing later in the disease course in disease severity score (1.7 ± 0.4 vs. 2.4 ± 0.6, P = 0.01), length of diagnosis delay (7.2 ± 6.4 vs. 2.3 ± 3.3 years, P=0.037), age of FMF onset (24.8 ± 19.9 vs. 5.6 ± 5.7 years of age, P=0.014) and rate of homozygosity to the M694V mutation (14.3% vs. 68.7% respectively). ELE traits in the study and control groups were alike. Conclusions FMF with ELE as the first disease manifestation form an uncommon subgroup, clinically and genetically diverging from the rest of the FMF-ELE patients.
No preview · Article · Jan 2012 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: Pemphigus vulgaris (PV) is a life-threatening disease affecting skin and mucous membranes. The "epitope spreading" theory posits that uncontrolled PV can gradually worsen because of exposure of cellular antigens to the immune system. To this end, high-dose systemic corticosteroids have been advocated as first-line treatment for patients with PV to achieve disease control.
To determine whether the initial dose of prednisone stratified by disease severity affects long-term disease severity.
A retrospective study was conducted on 58 patients with PV with at least five years of follow-up from diagnosis. Patients were categorized into three groups according to the initial dose of prednisone treatment. Parameters analyzed included age, gender, disease severity at baseline and follow-up, hospitalizations, prednisone doses and adjuvant therapy at follow-up, and remission rate.
Ten patients received initial low-dose prednisone or were treated initially without systemic CS, 19 patients received intermediate-dose prednisone, and 29 received high-dose prednisone. Disease severity at presentation correlated directly with initial prednisone doses. The duration of the first hospitalization and number of hospitalization days during the five-year follow-up period were significantly lower in the group treated with initial low-dose prednisone and similar for the groups treated with intermediate and high doses.
Disease severity of PV at presentation is a good predictor of the clinical course. Stratifying initial prednisone dose according to PV disease severity at presentation is appropriate.
No preview · Article · Aug 2011 · International journal of dermatology
[Show abstract][Hide abstract] ABSTRACT: Lentigines are a common pigmentary disorder in adults and in patients treated by psoralen and ultraviolet A (PUVA) radiation. Their appearance following treatment with narrow-band ultraviolet B (NB-UVB) radiation has been reported in only two patients.
To describe the clinical and histological features of NB-UVB-induced lentigines their relation to dosimetry and the course of the eruption in patients with mycosis fungoides (MF).
The files of all patients with MF treated in our department in 2003-2010 were searched to identify those in whom lentigines appeared following monotherapy with NB-UVB radiation.
Of the 73 patients with early stage MF identified, 10 met the study criteria. Lentigines were detected in skin previously involved by MF in seven patients, and in both involved and uninvolved skin in three patients. They appeared during therapy in three patients, after a mean of 56 exposures (range 50-61), and several months (mean 7.8) following completion of treatment in seven patients, after a mean of 69 exposures (range 32-157). Histopathological study of lesions from five patients revealed basal hyperpigmentation relative to adjacent normal-looking skin. Two lesions had a slight increased number of normal-looking melanocytes on immunohistochemical staining with melanoma cocktail. One lesion had elongated rete ridges. The lesions persisted throughout follow-up (mean 26.7 months) in 8 patients.
Patients with MF treated with NB-UVB may acquire lentigines. As opposed to PUVA-induced lentigines which are a known common side-effect of long-term treatment, NB-UVB-induced lentigines are uncommon but appear earlier, even after a few months of treatment.
No preview · Article · Jul 2011 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: The treatment of pemphigus, an autoimmune bullous disease, is based on the combination of corticosteroids and adjuvant therapies, such as immunosuppressive drugs, anti-inflammatory drugs and immunomodulatory procedures, such as intravenous immunoglobulin and therapeutic plasma exchange (TPE).
This study aims to assess our experience with TPE as a steroid-sparing modality in moderate and severe intractable pemphigus patients.
A retrospective evaluation for all intractable pemphigus patients treated by TPE in a university-affiliated tertiary referral medical centre between the years 1998 and 2008. Treatment protocol included three TPE treatments weekly for 1-3 months, combined with monthly pulse therapy of dexamethasone and/or cyclophosphamide. Maintenance therapy was based on once/bi weekly TPE treatments or monthly intravenous immunoglobulin.
Seven patients were included in the study, four with severe pemphigus vulgaris and three with moderate disease. Six of the seven patients responded to TPE: Four patients (57%) achieved complete remission and two patients (28%) achieved partial remission on minimal therapy. Mild adverse effects related to TPE were observed in two patients and included dizziness and mild headache.
TPE is a well-tolerated effective steroid-sparing agent in recalcitrant pemphigus patients.
No preview · Article · Jan 2011 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: Burkholderia cepacia is a common environmental bacterium that is resistant to disinfectants, and therefore is often encountered as a hospital-acquired pathogen. We describe an outbreak of B. cenocepacia bacteremia among hospitalized oncology patients.
A matched case-control study and an extensive environmental investigation were conducted. Species were identified by RFLP of the amplified recA gene. DNA was fingerprinted by pulsed-field gel electrophoresis (PFGE).
Between November 2005 and September 2006, B. cenocepacia bacteremia developed in 17 patients with underlying malignancy of whom 14 had tunneled central venous catheters. All patients had fever and chills which subsided following removal of the central catheter and administration of ceftazidime. Extensive epidemiological investigation could not find a common source for the outbreak. Patients were hospitalized in three different buildings with different health care personnel. Medications were prepared in different sites by different personnel. A multivariate analysis demonstrated that the independent risk factors for developing nosocomial B. cenocepacia bacteremia were hospitalization at the center for long-term support (OR 28.8; 95% CI 1.83-453.4) and reduced use of antibiotics during the last month (OR 0.07; 95% CI 0.01-0.40). All isolates had identical antimicrobial susceptibility; PFGE indicated that a complex of closely related strains was involved in the outbreak. All isolates were identified as B. cenocepacia, known to infect cystic fibrosis patients. Strict infection control measures terminated the outbreak.
B. cenocepacia is an emerging nosocomial pathogen among oncology patients.
[Show abstract][Hide abstract] ABSTRACT: Pancreatic panniculitis is a rare complication of carcinoma of the pancreas, most often accompanying the rare acinar cystadenocarcinoma. It presents with painful erythematous subcutaneous nodules typically located on the leg. We present a case of a 79-year-old man with neuroendocrine carcinoma of the pancreas and liver metastasis, who developed painful subcutaneous nodules on his shins. Laboratory values included a raised lipase level with normal amylase level and peripheral eosinophilia. The patient was treated with nonsteroidal anti-inflammatory drugs, dexamethasone and antibiotics, with resolution of the dermatological symptoms.
No preview · Article · Jul 2009 · Clinical and Experimental Dermatology
[Show abstract][Hide abstract] ABSTRACT: Atopic dermatitis (AD) is a common inflammatory skin disease. Methotrexate (MTX) was suggested as an effective treatment option in cases of moderate-to-severe atopic dermatitis. This study assessed the efficacy and safety of treatment with low weekly doses of methotrexate for moderate-to-severe AD in adults.
Twenty adult patients with moderate-to-severe AD were included in this retrospective study. Those patients were unresponsive to topical treatments, antihistamines and at least one of the second-line treatments. MTX in low weekly doses of 10-25 mg was administered orally or intramuscularly with folic acid supplementation 5 mg per week for at least 8-12 weeks. The response to treatment was evaluated by change in SCORAD (SCORing Atopic Dermatitis), DLQI (Dermatology Quality of Life Index) and the global assessment of the clinical response score.
After 8-12 weeks of treatment, we observed an objective response in most patients. There were 16 responders and 4 non-responders. The mean SCORAD and DLQI decreased by 28.65 units (44.3%) and 10.15 units (43.5%), respectively. The first improvement was observed after a period ranging from 2 weeks to 3 months (mean 9.95 w +/- 3.17). Treatment was more effective in adult onset AD than in childhood onset. Tolerance of treatment was good. However, nausea and an increase of liver enzymes were observed in 5 patients and 3 of them required a transient discontinuation of MTX. One patient developed peripheral neuropathy, which was resolved several weeks after the discontinuation of MTX.
MTX seems to be an effective and safe second-line treatment for patients with moderate-to-severe atopic dermatitis. A randomized, controlled study is warranted.
No preview · Article · Jul 2009 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: Gross migration of silicone gel from ruptured breast implants is a rare event. It is associated with extravasation of gel into the breast parenchyma, and to distant locations such as the abdominal wall and inguinal areas. This silicone deposits present as subcutaneous nodules and cause a local reaction known as siliconoma. We evaluated a 56-year-old woman who presented with a 2-year history of painful, firm and ill-defined subcutaneous nodules on the medial aspect of the shins and ankles. Her medical history was notable for bilateral breast augmentation with silicone implants 30 years before presentation. Although there were no signs or symptoms on breast examination, ultrasonography and magnetic resonance imaging confirmed that both implants had ruptured. Histological examination of a punch biopsy from a nodule on the shin found lobular granulomatous panniculitis. An excisional biopsy of the lesion was analysed by scanning electron microscopy and was found to contain silicone. This is a rare case of gross migration of silicone to the shins, originating from ruptured breast implants. To our knowledge, there is no previous report of silicone migration to such a distant location. We discuss the common presentation of silicone migration and highlight the importance of awareness among dermatologists and plastic surgeons about this unusual occurrence.
No preview · Article · Jun 2009 · Clinical and Experimental Dermatology
[Show abstract][Hide abstract] ABSTRACT: Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature.
To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group.
We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007.
Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16.6 years (range 16-17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission.
In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.
No preview · Article · Apr 2009 · British Journal of Dermatology
[Show abstract][Hide abstract] ABSTRACT: Cutaneous leishmaniasis is endemic in Israel. Leishmania major is the most prevalent species that cause cutaneous leishmaniasis. Current treatment options are limited and there are few investigations in search of alternative ones.
This study aims to assess our experience with intralesional sodium stibogluconate (SSG) in the treatment of cutaneous leishmaniasis.
A retrospective evaluation for all adult cases of cutaneous leishmaniasis treated by intralesional and intravenous SSG (Pentostam, GlaxoSmithKline) between 2004 and 2006 was performed, for cases referred to a tertiary care university-affiliated medical centre in Israel. Intralesional SSG was injected at 0.5 mL per lesion (50 mg). Treatment was repeated every 2-3 weeks for a total of 12 weeks. Intravenous SSG was administered at a dose of 20 mg/kg for 10-20 days.
Thirty-three cases of cutaneous leishmaniasis were treated with intralesional SSG during the study period. The patients consist of 26 males and 7 females, mostly Israeli military personnel, and there were a total of 93 lesions. Within 3 months from treatment onset, 91% (30/33) had completed healing of the cutaneous lesions after an average of 3 treatments (range 1-6). Side-effects were mild and were mostly pain during injection, with two patients developing mild local site reaction after the injection.
Intralesional SSG treatment is safe, effective and well tolerated with minimal side-effects.
No preview · Article · Mar 2009 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: Currently, psoriasis is thought to be an inflammatory response to an antigenic stimulation, in which angiogenesis plays a fundamental role. Very late antigen-1 (VLA-1) is a beta(1) integrin collagen receptor that is up-regulated in many angiogenic processes. Data on its role in psoriasis are sparse.
In a prospective study, we evaluated the staining of VLA-1 in lesional skin from patients with psoriasis and atopic dermatitis.
Frozen sections from skin biopsies of patients with chronic plaque-type psoriasis (n = 18) and chronic atopic dermatitis (n = 7) were stained with a monoclonal antibody to VLA-1. The number of blood vessels stained with VLA-1 and the staining intensity were evaluated. These were correlated with the histologic features.
The absolute number of blood vessels was found to be similar in the atopic and psoriatic samples. However, the number of vessels stained with anti-VLA-1, as well as the staining intensity, was shown to be significantly higher in the psoriasis group (P < 0.05). Differences between psoriatic lesions showing typical histological features of psoriasis and those showing features that overlap with dermatitis were found as well.
Expression of VLA-1 was found significantly higher in lesional dermal blood vessels of psoriatic patients compared with atopic patients. These findings suggest a possible role for VLA-1 in the pathological angiogenesis of psoriasis. It may be an additional tool for establishing the diagnosis of psoriasis and provide a basis for new strategies in the treatment of psoriasis.
No preview · Article · Mar 2008 · Journal of the European Academy of Dermatology and Venereology
[Show abstract][Hide abstract] ABSTRACT: The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual. Most descriptions are isolated case reports and case series are strikingly sparse.
To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms.
Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period.
Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma). No case of Hodgkin's lymphoma was found. In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first. The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years). Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB). Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy. Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed. In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy. One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled.
This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy. In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm. The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases. It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features.
No preview · Article · Sep 2006 · British Journal of Dermatology
[Show abstract][Hide abstract] ABSTRACT: Pityriasis lichenoides comprises a clinical and pathological spectrum of disorders. So far no highly effective treatment has been reported. Previous small studies have suggested that ultraviolet B (UVB) is a good alternative.
This is a retrospective analysis of 29 pityriasis lichenoides patients treated in our institution with broad- or narrow-band UVB during the period 1996-2002. Twenty-one of these patients had one or more previous unsuccessful treatments.
Complete response was achieved in 93.1% in both treatment groups, with 73% of them still relapse free after a mean follow-up of 58 and 38 months in broad- and narrow-band UVB treatment groups, respectively. Mild side-effects were observed in about one-third of the patients.
We believe both forms of UVB are a good option for pityriasis lichenoides and should be considered as the first line in generalized cases interested in treatment.
No preview · Article · Jun 2006 · Journal of the European Academy of Dermatology and Venereology