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1.
N Engl J Med ; 384(24): 2295-2305, 2021 06 17.
Artigo em Inglês | MEDLINE | ID: mdl-34097368

RESUMO

BACKGROUND: Rituximab and mycophenolate mofetil are used to treat pemphigus vulgaris, but they have not been adequately compared in clinical trials. METHODS: In a randomized, controlled trial, we assigned patients with moderate-to-severe pemphigus vulgaris in a 1:1 ratio to receive intravenous rituximab (1000 mg on days 1, 15, 168, and 182) or oral mycophenolate mofetil (2 g per day), in addition to an oral glucocorticoid administered on the same tapering schedule in the two groups. The primary end point was sustained complete remission at week 52, defined as the healing of lesions with no new active lesions, as reflected by a Pemphigus Disease Area Index (PDAI) activity score of 0 (on a scale of 0 to 250, with higher scores indicating greater disease severity), for at least 16 weeks without the use of glucocorticoids. Secondary end points were the cumulative dose of glucocorticoids, the number of disease flares, and the change from baseline in the score on the Dermatology Life Quality Index (DLQI; scores range from 0 to 30, with higher scores indicating greater impairment). RESULTS: Of the 135 patients who underwent randomization, 67 were assigned to receive rituximab and 68 to receive mycophenolate mofetil. The primary outcome was assessed in the modified intention-to-treat population: 62 patients in the rituximab group and 63 in the mycophenolate mofetil group. The median PDAI activity scores at baseline were 22.7 in the rituximab group and 18.3 in the mycophenolate mofetil group. At week 52, sustained complete remission was observed in 25 patients (40%) in the rituximab group and in 6 (10%) in the mycophenolate mofetil group (difference, 31 percentage points; 95% confidence interval [CI], 15 to 45; P<0.001). The mean cumulative glucocorticoid dose during the 52-week treatment period was 3545 mg in the rituximab group and 5140 mg in the mycophenolate mofetil group (difference, -1595 mg; 95% CI, -2838 to -353; P<0.001). There were 6 disease flares in the rituximab group and 44 in the mycophenolate mofetil group (adjusted rate ratio, 0.12; 95% CI, 0.05 to 0.29; P<0.001). The mean change in DLQI score was -8.87 points and -6.00 points, respectively (difference, -2.87 points; 95% CI, -4.58 to -1.17; P = 0.001). Serious adverse events occurred in 15 of 67 patients (22%) in the rituximab group and in 10 of 68 (15%) in the mycophenolate mofetil group. CONCLUSIONS: Rituximab was superior to mycophenolate mofetil in producing sustained complete remission at 52 weeks in patients with pemphigus vulgaris. Rituximab resulted in a greater reduction in glucocorticoid use than mycophenolate mofetil, but more patients in the rituximab group had serious adverse events. Further trials are needed to determine the comparative efficacy and safety of rituximab and mycophenolate mofetil beyond 52 weeks of treatment. (Funded by F. Hoffmann-La Roche; PEMPHIX ClinicalTrials.gov number, NCT02383589.).


Assuntos
Ácido Micofenólico/uso terapêutico , Pênfigo/tratamento farmacológico , Rituximab/uso terapêutico , Administração Oral , Adulto , Idoso , Método Duplo-Cego , Quimioterapia Combinada , Feminino , Glucocorticoides/administração & dosagem , Humanos , Análise de Intenção de Tratamento , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Indução de Remissão , Rituximab/efeitos adversos
2.
Contact Dermatitis ; 2024 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-38923529

RESUMO

BACKGROUND: Tefillin are a religious article worn by Jewish men during daily prayer. Tefillin dermatitis secondary to potassium dichromate sensitivity is recognised, but data remain sparse. OBJECTIVE: To investigate the prevalence and clinical characteristics of tefillin dermatitis. METHODS: Patients who underwent patch testing with the European baseline series in a tertiary dermatology clinic in 2009-2023 and were diagnosed with tefillin dermatitis were identified by file review and their clinical data recorded. RESULTS: Of 1679 consecutive male patients tested, 25 (1.49%) were diagnosed with tefillin dermatitis, accounting for 15.34% of all potassium-dichromate-positive patients (163/1679). Mean pre-symptomatic duration of tefillin use was 38 ± 16.9 years, and mean follow-up time, 3.1 ± 2.9 years. Patients presented with an eczematous rash on body areas in direct contact with the leather box or straps of the tefillin. An id reaction was noted in 32%, and sensitivity to other leather accessories, in 44%. Fourteen patients (56%) switched to chromate-free tefillin: symptoms resolved completely in 11 (79%) and partially in 2. LIMITATIONS: Retrospective cohort design. CONCLUSION: This is the largest study to date of tefillin dermatitis caused by sensitivity to potassium dichromate used in leather production. Prognosis after switching to chromate-free tefillin was good-to-excellent. Tefillin dermatitis may be more prevalent than previously thought.

3.
Isr Med Assoc J ; 26(5): 283-288, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38736342

RESUMO

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease involving apocrine gland-bearing regions. There is an under-representation of non-Caucasians in epidemiologic studies of HS. The characteristics of HS in Israeli Arabs have not yet been studied. OBJECTIVES: To investigate the demographic and clinical profile of HS in the Israeli Arab population. METHODS: A retrospective analysis was conducted in two cohorts of patients with HS in Israel. The patients were derived from the database of a large health management organization (n=4191, 639 Arabs; population-based) and a major tertiary medical center (n=372, 49 Arabs). Demographic and clinical data were compared between ethnic groups. RESULTS: The prevalence of HS in Israeli Arabs was found to be 0.5%, fivefold higher than in Jews. Arab patients were younger (35.3 vs. 40.5 years, P < 0.001) and mostly male (52% vs. 35.7%, P < 0.001), with lower rates of co-morbidities, including smoking (40.8% vs. 55.7%, P < 0.001), hyperlipidemia, and depression as well as a higher rate of dissecting cellulitis (10.2% vs. 1.9%, P = 0.008). HS was more severe in Arabs, but of shorter duration, with mainly axillary involvement (79.6% vs. 57.9%, P = 0.004). Treatment with hormones was more common in Jews, and with biologic agents in Arabs. CONCLUSIONS: The findings suggest a different phenotype of HS in Arabs, warranting further study.


Assuntos
Árabes , Hidradenite Supurativa , Judeus , Humanos , Hidradenite Supurativa/etnologia , Hidradenite Supurativa/epidemiologia , Árabes/estatística & dados numéricos , Judeus/estatística & dados numéricos , Israel/epidemiologia , Masculino , Feminino , Adulto , Estudos Retrospectivos , Prevalência , Pessoa de Meia-Idade , Comorbidade , Estudos de Coortes
4.
Dermatol Ther ; 35(5): e15397, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35194896

RESUMO

Rituximab is the front-line therapy for pemphigus disease. Although very effective, relapse rates are high. We assessed factors associated with disease remission and early relapse following the first rituximab cycle. A single center, retrospective cohort study of patients with pemphigus treated with rituximab (1000 mg 0, 14 days) at the Autoimmune Bullous Disease Clinic of the Division of Dermatology in Rabin Medical Center, Israel, between January 1, 1995 and March 31, 2020. The cohort included 99 patients with a median follow-up of 37 months (range 12-155). After a single rituximab cycle, 74 patients (75%) achieved remission. Increased time to rituximab was associated with decreased remission rates (OR, 0.98 per month; 95% CI, 0.97-0.998). Of patients in remission with sufficient follow-up, 15/69 (22%) experienced an early relapse (≤12 months from remission). Prolonged time to rituximab and increased baseline disease severity, were associated with early relapse (OR, 1.02 per month; 95% CI, 1.001-1.04; OR, 1.04 per point; 95% CI, 1.01-1.08, accordingly). Initiating rituximab early following diagnosis is recommended. Maintenance rituximab infusions, especially for patients with severe baseline disease, should be further investigated.


Assuntos
Doenças Autoimunes , Pênfigo , Estudos de Coortes , Humanos , Fatores Imunológicos/efeitos adversos , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Recidiva , Estudos Retrospectivos , Rituximab/efeitos adversos , Resultado do Tratamento
5.
Lasers Med Sci ; 37(7): 2899-2905, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35412157

RESUMO

Actinic keratoses are common cutaneous lesions with a potential to progress to invasive squamous cell carcinoma. Therefore, treatment is crucial. The Tixel® is a noninvasive thermomechanical device designed to transfer heat to the upper dermis in a controlled manner according to a predetermined setting. This study aimed to evaluate the safety and efficacy of a thermomechanical fractional skin resurfacing technology for the treatment of facial and scalp actinic keratoses. A prospective, open-label, before-after study was conducted in a tertiary medical centre from May 2020 to April 2021. Patients presenting with facial/scalp actinic keratoses of mild-to-moderate thickness underwent 2 or 3 Tixel treatments (depending on clinical improvement), 3-4 weeks apart. The reduction in lesion count and overall improvement in appearance were assessed by clinical examination and digital photography. Findings were compared between baseline and follow-up at 3 months after the last treatment session. Patient satisfaction was evaluated by questionnaire, and adverse effects were documented. A total of 20 patients participated in the study. All completed 2-3 treatments and follow-up visits. Assessment of digital photographs was performed by 2 assessors blinded to the timepoint at which each photo was taken (before or after treatment). The average number of lesions at baseline was 9.8 (± 4.8) and the mean reduction in lesion count was 7.9 (± 4.4) (80.6%). Complete clearance was observed in 31.6% of patients. No adverse effects were noted during treatment and follow-up. Most patients reported being "very satisfied" or "satisfied" with the treatment results (85%) and experience (95%). Treating facial and scalp actinic keratoses with the Tixel device was found to be effective and safe.


Assuntos
Ceratose Actínica , Humanos , Ceratose Actínica/tratamento farmacológico , Estudos Prospectivos , Rejuvenescimento , Couro Cabeludo/patologia , Pele/patologia , Resultado do Tratamento
6.
Dermatol Ther ; 34(1): e14637, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33278051

RESUMO

Cutaneous viral warts (CVW), caused by human papillomavirus, often have a self-limited course. However, some patients experience a recalcitrant disease despite treatment. Retinoids are considered the mainstay of therapy in many dermatologic diseases. Data on their use for viral warts are limited. To systematically review the published evidence on the efficacy and safety of retinoids for the treatment of CVW. A systematic review and meta-analysis of topical or systemic retinoid treatment for CVW was performed in accordance with the PRISMA statement. The primary outcome was clinical response; secondary outcomes were recurrence rate and adverse events. Fourteen publications including 399 patients treated exclusively with retinoids (65% topical, 35% systemic) were evaluated. The complete response rate was 64% (95% CI, 46-78%; I2 =80%) for topical treatment and 61% (95% CI, 44-76%; I2 =69%) for systemic treatment. The most common side effects were irritant contact dermatitis and cheilitis, respectively. Relapse rates were 6% and 17%, respectively. The reviewed studies were considerably heterogenous and most lacked a control group. Both topical and systemic retinoids are effective and safe as monotherapy for CVW. Further studies are required to determine their exact role in this setting.


Assuntos
Retinoides , Verrugas , Administração Cutânea , Administração Tópica , Humanos , Recidiva Local de Neoplasia , Retinoides/efeitos adversos , Resultado do Tratamento , Verrugas/diagnóstico , Verrugas/tratamento farmacológico
7.
Dermatology ; 237(3): 450-456, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33567432

RESUMO

BACKGROUND: A combined regimen of rituximab with corticosteroids for the treatment of pemphigus was effective in a prospective randomized controlled trial. OBJECTIVE: To assess real-life response to rituximab in patients with pemphigus. METHODS: A retrospective cohort of patients with pemphigus treated with ≥1 rituximab cycles (1,000 mg on days 0 and 14). The primary outcome was remission rate after 1 cycle. For efficacy analyses, a minimal 6-month follow-up was required. Adverse events were assessed in all patients. RESULTS: The cohort included 117 patients for safety analysis, 108 for efficacy analysis (median follow-up of 33 months). All but one received concomitant corticosteroids, a third also received adjuvants. Overall, 80/108 patients (74%) achieved remission after the first rituximab cycle at a median of 5.5 months. Relapses occurred in 39 patients (49%) at a median of 18 months. Repeating treatment in relapsed patients increased remission rates to 75 and 88% after the second and third cycles, respectively. Adverse events were similar to those of previous publications. Two elderly patients died of infections attributable to rituximab combined with high-dose corticosteroids. CONCLUSION: In a large real-life long-term cohort, rituximab with corticosteroids ± adjuvants induced remission in most patients with pemphigus, with relatively favorable safety. Repeating treatment following relapse or remission failure was beneficial.


Assuntos
Fatores Imunológicos/uso terapêutico , Pênfigo/tratamento farmacológico , Rituximab/uso terapêutico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Israel , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
8.
Dermatology ; 237(2): 179-184, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-32756069

RESUMO

INTRODUCTION: In a randomized prospective trial, adjuvant rituximab was more efficacious than corticosteroids alone in the treatment of pemphigus; however, real-life data are limited. Rituximab treatment for pemphigus has only recently been introduced to the Israeli health basket. Previously, patients received rituximab if they paid out of pocket or through private insurance, separating patients into 2 treatment groups, mostly based on economic capability. METHODS: A retrospective cohort study of the 12-month clinical response of pemphigus vulgaris/foliaceus patients. We compared patients after a single cycle (1,000 mg on days 0 and 15 or weekly 375 mg/m2 for 4 weeks) of adjuvant rituximab with systemic corticosteroids ± steroid-sparing agents, to patients who were prescribed rituximab, could not obtain it, and received systemic corticosteroids ± steroid-sparing agents. RESULTS: Forty-five patients were included (adjuvant rituximab, n = 29; immunosuppression alone, n = 16). At baseline, rituximab patients had a higher mean pemphigus disease area index (PDAI) (p = 0.07) and higher mean daily dosages of prednisone (1.51 vs. 1.16 mg/kg, p = 0.39). All patients but 1 in the rituximab group continued systemic steroids, and 31% in the rituximab group versus 50% in the immunosuppression-alone group received systemic adjuvants. At 12 months, partial or complete remission rates (on or off maximum 40 mg/day prednisone equivalent) were nonsignificantly higher in the rituximab group (62 vs. 50%, p = 0.53); however, patients on rituximab showed faster remissions (3.4 ± 1.9 vs. 5.9 ± 3.6 months; p = 0.03) with a trend for a greater PDAI reduction (p = 0.051). Adverse events were comparable. CONCLUSIONS: In this real-life study, a single cycle of rituximab achieved more remissions and sooner compared to conventional immunosuppression, but the differences were not significant, probably due to a small sample size and severe baseline disease in the rituximab group. Future real-life studies on larger groups are needed.


Assuntos
Imunossupressores/uso terapêutico , Pênfigo/tratamento farmacológico , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Adulto , Azatioprina/uso terapêutico , Quimioterapia Combinada , Feminino , Humanos , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/uso terapêutico , Prednisona/administração & dosagem , Recidiva , Indução de Remissão , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo
9.
Dermatology ; 237(3): 389-395, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33279886

RESUMO

BACKGROUND: Genital warts, caused by the human papillomavirus, are a common sexually transmitted disease. The warts can regress spontaneously or exhibit a persistent clinical course. Various therapeutic modalities are available, yet none is curative, and there may be recurrences. Retinoids are considered the mainstay of therapy in many dermatologic diseases. Data on their use for genital warts are limited. OBJECTIVE: To systematically review the published evidence on the efficacy and safety of retinoids for the treatment of genital warts. METHODS: A systematic review and meta-analysis of all publications evaluating topical or systemic retinoids for the treatment of genital warts was performed. The primary outcome was complete response (CR); the secondary outcomes were recurrence rate and adverse events. RESULTS: Six publications were evaluated, three randomized controlled trials and three prospective cohort studies, including a total of 141 patients with genital warts treated exclusively with retinoids (90% with isotretinoin). CR rates were 100% for systemic etretinate (3 out of 3 patients, 95% CI 28-81%) and 56% for isotretinoin (95% CI 28-81%; I2 = 84%). Topical etretinate did not induce CR. The most common side effect of topical agents was irritant contact dermatitis (36%) and that of systemic agents mucocutaneous disorders (80%). The relapse rate was 12% for oral isotretinoin and was unavailable for the other modalities. CONCLUSIONS: Current data suggest that unlike topical retinoids, systemic retinoids are an effective and safe treatment for genital warts. Further studies are required to determine their specific role and the most effective regimen for each derivative.


Assuntos
Condiloma Acuminado/tratamento farmacológico , Retinoides/uso terapêutico , Administração Oral , Administração Tópica , Humanos
10.
Photodermatol Photoimmunol Photomed ; 37(5): 374-379, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33556208

RESUMO

BACKGROUND: Inherited genetic erythropoietic protoporphyria (EPP) is characterized by a photosensitive rash that emerges during infancy or early childhood. Acquired EPP can erupt at any age, even during adulthood, and is associated with hematological disorders. A third, less-studied type of EPP is also inherited but appears later in life (during adulthood). PURPOSE: To evaluate the characteristics of inherited genetic late-onset (IGLO) EPP. METHODS: A systematic comprehensive search of the literature was conducted using PubMed, Google Scholar, ScienceDirect, and clinicaltrials.gov databases. Studies describing patients with IGLO EPP were included. Additionally, we present an index case of a patient, treated at our clinic in whom inherited genetic EPP was diagnosed at age 21 years. RESULTS: The search yielded 1514 citations. Five publications were eligible for review. Along with our case, 7 patients (4 males) were included in the analysis. Mean age at disease onset was 34.2 years (range 18-69, median 30). Most patients presented with mild pruritus and rash in a photosensitive distribution. Mean level of free erythrocyte protoporphyrin IX (FEP) was 8.6 µmol/L. A mutant ferrochelatase gene (FECH) in trans to a hypomorphic FECH allele was found in 3 of the 4 patients who underwent genetic testing. CONCLUSION: We describe the distinct features of IGLO EPP. This work emphasizes that a diagnosis of inherited genetic EPP should not be ruled out in adults with new-onset photosensitive manifestations.


Assuntos
Transtornos de Fotossensibilidade , Protoporfiria Eritropoética , Adolescente , Adulto , Idoso , Alelos , Pré-Escolar , Ferroquelatase/genética , Ferroquelatase/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Transtornos de Fotossensibilidade/genética , Protoporfiria Eritropoética/genética , Adulto Jovem
11.
Photodermatol Photoimmunol Photomed ; 37(5): 431-438, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33738834

RESUMO

Solar urticaria is a well-recognized photodermatosis, sometimes accompanied by angioedema. However, isolated solar angioedema (ISA) is a rare and unrecognized entity. The purpose of our work was to systematically review the available data on ISA. Therefore, a systematic review of studies evaluating ISA was performed. Additionally, a case of a 21-years-old patient from our photodermatosis service is presented. The search yielded 421 publications, with 3 eligible for review. Together with our case, 5 cases were included overall. All patients were female. Four out of 5 patients first experienced ISA at childhood or early adulthood (age range 6-22 years). UVA photoprovocation was positive in the 3 out of the 4 patients who were tested. Improvement was noted following NB-UVB hardening (2 out of 5 patients) or a short course of oral prednisone (3 out of 5 patients) combined with regular sunscreen application. To conclude, ISA is an extremely rare entity, although it may be underdiagnosed due to lack of awareness. The clinician must consider ISA in the differential diagnosis of angioedema since it can have a detrimental effect on quality of life. Besides sun avoidance, there is no consensus regarding treatment.


Assuntos
Angioedema , Transtornos de Fotossensibilidade , Urticária , Adolescente , Adulto , Angioedema/diagnóstico , Angioedema/etiologia , Criança , Feminino , Humanos , Transtornos de Fotossensibilidade/diagnóstico , Transtornos de Fotossensibilidade/etiologia , Qualidade de Vida , Luz Solar/efeitos adversos , Adulto Jovem
12.
Lasers Med Sci ; 36(9): 1909-1916, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33580846

RESUMO

The pulsed dye laser (PDL) is the standard treatment for port-wine stains (PWS). Maximal improvement occurs after multiple treatment sessions; however, the optimal treatment interval has yet to be determined. The aim of this study was to review whether there is an association between PDL treatment interval and outcome of PWS. Six databases were searched by three reviewers for publications investigating treatment of PWS with PDL. The 75% improvement rates (75IR) were extracted for quantitative analysis. Meta-regression was used to investigate the association between treatment intervals and 75IR. The systematic review included 1 RCT and 33 cohort studies (7 prospective cohorts and 26 retrospective cohorts), with a total of 3777 patients. The pooled 75IR was 37% (95% CI 29-45%; I2 = 95%). Light Fitzpatrick skin type (p = 0.04), facial anatomic location (p = 0.01), and young age (p = 0.008) were associated with 75IR. In an unadjusted (p = 0.42) and multivariable adjusted (p = 0.98) meta-regression, no association was found between time interval between treatments and 75IR. These results persisted in a sensitivity analysis of studies with a mean patient age of ≤ 1. The majority of included studies were heterogeneous and retrospective. Based on cohort studies of low-to-moderate quality, time intervals between PDL treatments are not associated with PWS outcome.


Assuntos
Lasers de Corante , Mancha Vinho do Porto , Humanos , Lasers de Corante/uso terapêutico , Mancha Vinho do Porto/radioterapia , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
13.
J Am Acad Dermatol ; 82(3): 575-585.e1, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29438767

RESUMO

BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.


Assuntos
Fatores Imunológicos/administração & dosagem , Pênfigo/diagnóstico , Pênfigo/terapia , Plasmaferese , Guias de Prática Clínica como Assunto , Academias e Institutos/normas , Administração Intravenosa , Antígenos CD20/imunologia , Terapia Combinada/métodos , Terapia Combinada/normas , Consenso , Técnica Delphi , Dermatologia/métodos , Dermatologia/normas , Quimioterapia Combinada/métodos , Quimioterapia Combinada/normas , Europa (Continente) , Glucocorticoides/administração & dosagem , Humanos , Pênfigo/imunologia , Rituximab/administração & dosagem , Índice de Gravidade de Doença
14.
Dermatology ; 236(6): 493-499, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32289785

RESUMO

INTRODUCTION: Surgery is commonly regarded as the mainstay of treatment of extramammary Paget disease (EMPD); however, nonsurgical approaches have gained popularity in recent years. OBJECTIVES: To review the published evidence for the efficacy and safety of nonsurgical modes of therapy for EMPD. METHODS: A systematic review and meta-analysis of nonsurgical EMPD treatments was performed. The primary outcome was complete response (CR); secondary outcomes were clinical regression by ≥50%, adverse events, and recurrence rate. RESULTS: The systematic review included 43 observational studies (341 patients; 7 prospective cohort studies, 19 retrospective cohort studies, and 17 cases series) evaluating 5 treatment modalities. Imiquimod (13 studies, 110 patients) administered at variable doses ranging from daily to twice weekly for 2-56 weeks demonstrated CR of 54% (95% CI, 40-67%; I2 = 37%) and had a satisfactory safety profile. In 14 heterogeneous studies (122 patients) evaluating photodynamic therapy (PDT), only 36% (95% CI, 22-53%; I2 = 52%) of patients achieved CR. Radiotherapy (12 studies, 67 patients) showed CR of 97%, but was associated with local and systemic side effects. Ablative lasers and topical fluorouracil and calcipotriene lacked adequate evidence of efficacy. CONCLUSIONS: Imiquimod and radiotherapy are the most appropriate nonsurgical modalities for EMPD treatment given their good efficacy and safety profile. PDT has limited efficacy but may be appropriate in selected clinical settings.


Assuntos
Doença de Paget Extramamária/terapia , Humanos
15.
Harefuah ; 159(1): 31-33, 2020 Jan.
Artigo em Hebraico | MEDLINE | ID: mdl-31930805

RESUMO

INTRODUCTION: Pemphigus is a chronic autoimmune bullous disease. To date there is no curative treatment for the disease. The standard treatment has been based on systemic corticosteroids and immune-suppressants. Rituximab is a monoclonal antibody against CD20 cells which leads to B cell depletion, resulting in decreased antibody production. In recent years increasing evidence on promising efficacy and safety of rituximab in the treatment of pemphigus has emerged. This review presents the key publications and the Israeli experience with rituximab treatment at the Rabin and the Sheba Medical Centers. Disclosure: Prof. Daniel Mimouni participated in an advisory board of Roche.


Assuntos
Doenças Autoimunes , Fatores Imunológicos/uso terapêutico , Pênfigo/tratamento farmacológico , Rituximab/uso terapêutico , Anticorpos Monoclonais Murinos , Humanos , Imunossupressores
16.
J Am Acad Dermatol ; 81(5): 1120-1126, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30797849

RESUMO

BACKGROUND: Disorders of the umbilicus are commonly seen in infancy, including hernias, infections, anomalies, granulomas, and malignancies. Meticulous inspection of the umbilicus at birth might reveal a persisting embryonic remnant, such as an omphalomesenteric duct (OMD), manifested by a variety of cutaneous signs, such as an umbilical mass, granulation tissue, or discharge. OBJECTIVE: To systematically review the available data regarding the presence and management of OMD remnant with cutaneous involvement to suggest a practical approach for diagnosis and treatment. METHODS: A systematic review of the literature evaluating OMD anomalies presenting with cutaneous symptoms was performed. In addition, an index case of an 11-month-old patient is presented. RESULTS: We included 59 publications reporting 536 cases; 97% of the patients whose age was noted were infants (mean age 11 months). In 7.5% of the cases, diagnosis was established only after treatment failure. In 6.4% of patients, nonlethal complications were reported, and in 10.3%, the outcome was death, partly due to delayed diagnosis or mismanagement. LIMITATIONS: Limited quality of the collected data, reporting bias. CONCLUSION: OMD is relatively rare; however, the clinician must consider this remnant while examining patients with umbilical abnormalities because mismanagement could cause severe morbidity and mortality.


Assuntos
Dermatopatias/etiologia , Ducto Vitelino/anormalidades , Humanos , Lactente , Dermatopatias/patologia , Dermatopatias/terapia
17.
Isr Med Assoc J ; 21(9): 580-584, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31542900

RESUMO

BACKGROUND: Whole-body integrated positron emission tomography / contrast-enhanced computed tomography (PET/CT) scan is increasingly used in cutaneous lymphomas. However, the value of PET/CT in the detection of cutaneous lesions in primary cutaneous B-cell lymphoma (PCBCL) has barely been investigated. OBJECTIVES: To investigate the diagnostic accuracy of PET/CT in tracking cutaneous involvement in PCBCL. METHODS: A retrospective study was conducted on 35 consecutive patients diagnosed with cutaneous B-cell lymphoma according to the World Health Organization classification who were evaluated with PET/CT as the initial staging procedure before treatment. RESULTS: Thirty-five patients met the study criteria. In two patients extracutaneous disease was detected by PET/CT and CT and confirmed by biopsy. Of the 33 patients with PCBCL, 26 (79%) had small cell PCBCL (18 marginal-zone, 8 follicle-center lymphoma) and 7 (21%) had large cell PCBCL (3 follicle-center, 3 leg-type, 1 indeterminate). PET/CT detected skin lesions in 3 of 26 patients (12%) with small-cell PCBCL as compared to 6 of 7 patients with large-cell PCBLC (86%), a 7.4-fold detection risk (95% confidence interval, 2.4-22, P = 0.004). The PET-positive subgroup was characterized by larger lesion size (P < 0.001) and a higher Ki-67 proliferation index (P < 0.001). CONCLUSIONS: The sensitivity of PET/CT for detecting cutaneous involvement of lymphomas is low for small-cell PCBCL but high for large-cell types, and thus may facilitate therapeutic strategies.


Assuntos
Fluordesoxiglucose F18 , Linfoma de Células B/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Compostos Radiofarmacêuticos , Neoplasias Cutâneas/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade , Pele/diagnóstico por imagem , Adulto Jovem
18.
Lasers Med Sci ; 33(3): 597-601, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29299693

RESUMO

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Picosecond (PS) lasers are novel laser devices designated to target small chromophores. A side effect of these lasers is blistering due to epidermal-dermal separation. We aimed to harness this side effect of the PS lasers to treat patients with VEN. The purpose of this study was to report our experience treating VEN using a PS 532-nm laser. We present a retrospective case series of six patients with large VEN who were treated using a PS 532-nm laser (2-6 treatments, 8-10 weeks apart). Response in clinical photographs was assessed by two independent dermatologists and graded on a scale of 0 (exacerbation) to 4 (76-100% improvement). Patient satisfaction was recorded on a scale of 1-5. All patients demonstrated significant improvement. Average improvement was 3.7 on the quartile scale of improvement. Patient satisfaction rate averaged 4.7. The PS 532-nm laser is a promising novel modality for the treatment of large VEN.


Assuntos
Lasers de Estado Sólido/uso terapêutico , Nevo Sebáceo de Jadassohn/cirurgia , Nevo/cirurgia , Adolescente , Adulto , Feminino , Humanos , Masculino , Satisfação do Paciente , Estudos Retrospectivos
19.
J Am Acad Dermatol ; 77(2): 256-260, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28495498

RESUMO

BACKGROUND: Venous thromboembolism (VTE) has been reported to be a significant cause of death in patients with pemphigus. OBJECTIVE: We sought to assess the incidence and characteristics of VTE in patients with pemphigus. METHODS: A retrospective study following a cohort of 172 patients with newly diagnosed pemphigus for the development of VTE was conducted. RESULTS: Over a mean follow-up time of 4 years, 10 patients (6%) had development of VTE at a median of 4 months from pemphigus diagnosis. The highest risk was found in the first year, at 5 VTE events per 100 patient-years or a 5% VTE risk for that first year. Five patients had deep vein thromboses, 4 had pulmonary embolisms, and 1 had both. Age and gender were not related to the VTE risk. Most patients had VTE risk factors, either hereditary or acquired as the result of pemphigus treatments and their complications, including hospitalization, immobilization, and infections. LIMITATIONS: The use of existing health records limited the assessment of asymptomatic VTE and VTE risk factors; a matched control population was not studied. CONCLUSIONS: VTEs are not rare in patients with pemphigus, more so in the first year after diagnosis. VTE risk must be assessed in all patients, especially when hospitalized, and thrombo-prophylaxis should be initiated in qualifying cases.


Assuntos
Pênfigo/diagnóstico , Embolia Pulmonar/epidemiologia , Tromboembolia Venosa/epidemiologia , Trombose Venosa/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Hospitalização , Humanos , Imobilização , Incidência , Infecções/etiologia , Masculino , Pessoa de Meia-Idade , Pênfigo/complicações , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Adulto Jovem
20.
J Am Acad Dermatol ; 77(4): 691-699, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28645646

RESUMO

BACKGROUND: Studies evaluating whether malignancy rate is increased in patients with bullous pemphigoid (BP) have reached conflicting results. OBJECTIVE: We sought to determine whether BP is associated with malignancy. METHOD: Medline, EMBASE, the Cochrane library, and reference lists of included studies were searched for comparative studies that evaluated the relationship between BP and malignancy. Data were analyzed on the basis of study design: cross-sectional, case control, and cohort. A meta-analysis was performed by using a random effects model to estimate pooled odds ratio. RESULTS: The review included 8 studies. No association between BP and overall cancer was found for any of the study designs. Although a single cohort study reported an association with lymphoid leukemia and kidney and larynx cancer, a pooled analysis of case-control studies did not. A pooled analysis of cross-sectional studies found a significant association between BP and hematologic malignancies. LIMITATIONS: The paucity of well-designed studies hindered the possibility of proving or disproving the BP-cancer association. CONCLUSION: We did not find an association of BP with overall malignancy, but a possible association with hematologic malignancy was observed.


Assuntos
Neoplasias/epidemiologia , Penfigoide Bolhoso/epidemiologia , Estudos de Casos e Controles , Estudos de Coortes , Estudos Transversais , Neoplasias Hematológicas/epidemiologia , Humanos
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