RESUMEN
BACKGROUND: Atopic diseases are characterized by IgE antibody responses that are dependent on cognate CD4 T cell help and T cell-produced IL-4 and IL-13. Current models of IgE cell differentiation point to the role of IgG memory B cells as precursors of pathogenic IgE plasma cells. The goal of this work was to identify intrinsic features of memory B cells that are associated with IgE production in atopic diseases. METHODS: Peripheral blood B lymphocytes were collected from individuals with physician diagnosed asthma or atopic dermatitis (AD) and from non-atopic individuals. These samples were analyzed by spectral flow cytometry, single cell RNA sequencing (scRNAseq), and in vitro activation assays. RESULTS: We identified a novel population of IgG memory B cells characterized by the expression of IL-4/IL-13 regulated genes FCER2/CD23, IL4R, IL13RA1, and IGHE, denoting a history of differentiation during type 2 immune responses. CD23+ IL4R+ IgG+ memory B cells had increased occurrence in individuals with atopic disease. Importantly, the frequency of CD23+ IL4R+ IgG+ memory B cells correlated with levels of circulating IgE. Consistently, in vitro stimulated B cells from atopic individuals generated more IgE+ cells than B cells from non-atopic subjects. CONCLUSIONS: These findings suggest that CD23+ IL4R+ IgG+ memory B cells transcribing IGHE are potential precursors of IgE plasma cells and are linked to pathogenic IgE production.
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Células B de Memoria , Receptores de IgE , Humanos , Receptores de IgE/metabolismo , Interleucina-13 , Interleucina-4 , Inmunoglobulina E , Inmunoglobulina G , Subunidad alfa del Receptor de Interleucina-4 , Lectinas Tipo CRESUMEN
Methotrexate is historically recognized as an effective treatment of pemphigus but its utility as a single or alternate steroid-sparing agent was not recognized in recent consensus recommendations in pemphigus management. We aimed to evaluate the efficacy and safety of a treatment course for pemphigus that involves methotrexate as a single or steroid-sparing agent. In a retrospective cohort study, we examined patients with pemphigus vulgaris or pemphigus foliaceus who were on ≥3 months of methotrexate therapy. Efficacy and safety were evaluated by established pemphigus disease endpoints. Of the 34 patients who met inclusion criteria, 25 (73.5%) were on glucocorticoids at time of methotrexate initiation (median follow-up: 5.4 years; median time on methotrexate: 3.7 years). An appreciable proportion achieved disease control (91.2%), with some achieving clinical remission off all systemic therapies (23.5%). For patients on glucocorticoids, median time to control was 42 days, median time to minimal steroid dose tapering (5 mg prednisone) was 161 days, and median time to complete steroid tapering was 308 days. For patients on methotrexate as a single agent, median time to control was 119 days. Among all patients, relapse commonly occurred (88.2%). At last follow-up, 26.5% were managed on topical therapies alone and 11.8% required systemic steroid therapy. Methotrexate was largely tolerated with a low incidence of adverse events leading to treatment discontinuation (2.9%). Methotrexate has the potential to be an effective and well-tolerated option for patients and may be considered for use as an alternate single or steroid-sparing agent for pemphigus.
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Pénfigo , Glucocorticoides , Humanos , Metotrexato/efectos adversos , Pénfigo/inducido químicamente , Pénfigo/diagnóstico , Pénfigo/tratamiento farmacológico , Prednisona/uso terapéutico , Inducción de Remisión , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The first-line treatment for patients with bullous pemphigoid (BP), the most common autoimmune blistering disease, is systemic glucocorticoids, which are associated with numerous side effects. Mycophenolate mofetil (MMF) may be beneficial in BP as a steroid-sparing alternative; however, evidence is limited. OBJECTIVES: To evaluate the efficacy and safety of MMF in patients with BP. METHODS: In this retrospective chart review, records of patients with BP treated with MMF alone or in combination with prednisone, who presented between 2013 and 2017, were analyzed. RESULTS: Twenty-six patients were included. Twelve patients were treated with MMF alone (monotherapy) and fourteen were treated with MMF and prednisone concomitantly at some point during their treatment course (dual therapy). Improvement in BP was observed in 26 (100%) patients with MMF therapy. Mean time to improvement was 0.8 months. Twenty-five (96.2%) patients [11/12 (91.7%) on monotherapy and 14/14 (100%) on dual therapy] achieved complete control of their disease. Mean time to complete control amongst all patients was 5.6 months. Twelve (46.2%) patients [4/12 (33.3%) on monotherapy and 8/14 (57.1%) on dual therapy] experienced disease remission with no subsequent flares for up to 15 months after MMF was discontinued. Twelve mild adverse effects were reported with one individual discontinuing therapy due to gastrointestinal symptoms. No serious adverse effects were reported. CONCLUSION: MMF is a safe and effective therapy for BP and can yield improvement and complete response in most patients and remission in some. J Drugs Dermatol. 2022;21(2):151-155. doi:10.36849/JDD.6042.
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Ácido Micofenólico , Penfigoide Ampolloso , Quimioterapia Combinada , Humanos , Inmunosupresores/efectos adversos , Ácido Micofenólico/efectos adversos , Penfigoide Ampolloso/diagnóstico , Penfigoide Ampolloso/tratamiento farmacológico , Prednisona/uso terapéutico , Estudios Retrospectivos , Resultado del TratamientoAsunto(s)
Actitud Frente a la Salud , Baile , Hospitalización , Medicina en las Artes , Humanos , MasculinoRESUMEN
Darier disease is an autosomal dominant genodermatosis of abnormal keratinization characterized by hyperkeratotic papules and plaques with a predilection for seborrheic areas. We report a case of a rare vesiculobullous variant of treatment-resistant Darier disease in a 55-year-old woman that failed topical tacrolimus and topical and oral glucocorticoids. Cetirizine was initiated at 10 mg daily and increased to 40 mg daily over four weeks, with resultant marked improvement of the patient's burning sensation. A punch biopsy revealed a perivascular infiltrate of eosinophils. This patient's symptomatic improvement with cetirizine, which has antagonizing properties against eosinophils, highlights the potential role of eosinophils in the pathogenesis of vesiculobullous Darier disease. We suggest that major basic protein secreted by eosinophils may propagate blister formation in vesiculobullous Darier disease by disrupting desmosomes. J Drugs Dermatol. 2019;18(2):213-214.
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Antialérgicos/uso terapéutico , Cetirizina/uso terapéutico , Enfermedad de Darier/tratamiento farmacológico , Enfermedades Cutáneas Vesiculoampollosas/tratamiento farmacológico , Antialérgicos/farmacología , Cetirizina/farmacología , Enfermedad de Darier/complicaciones , Enfermedad de Darier/diagnóstico , Relación Dosis-Respuesta a Droga , Eosinófilos/efectos de los fármacos , Femenino , Humanos , Persona de Mediana Edad , Enfermedades Cutáneas Vesiculoampollosas/complicaciones , Enfermedades Cutáneas Vesiculoampollosas/diagnóstico , Resultado del TratamientoRESUMEN
Importance: The first-line treatment for patients with chronic spontaneous urticaria (CSU), which is divided into idiopathic and autoimmune subtypes, consists of H1-antihistamines. However, limited evidence guides the treatment of CSU after maximal therapy with antihistamines fails. Two randomized clinical trials suggest that dapsone may be a successful second-line therapy. Objective: To evaluate the efficacy and safety of dapsone therapy in patients with CSU. Design, Setting, and Participants: This retrospective medical record review included 79 patients with CSU treated with dapsone who presented to the tertiary care academic medical center at the New York University School of Medicine, New York, New York, from January 1, 2005, through April 15, 2017. Follow-up was completed on February 28, 2018. Data were analyzed from March 1 through May 31, 2018. Exposures: Treatment with oral dapsone for CSU. Main Outcomes and Measures: Efficacy of dapsone therapy for CSU was evaluated as improvement, complete response, and remission. Results: Seventy-nine patients (65% women; mean [SD] age, 49.8 [16.1] years [range, 20-79 years]) were included in the analysis. Forty-five patients had chronic idiopathic urticaria and 34 had chronic autoimmune urticaria. Improvement in CSU was observed in 62 patients (78%) (36 [80%] with idiopathic and 26 [76%] with autoimmune disease) with dapsone. Mean (SD) time to improvement was 1.1 (1.0) months. A complete response was achieved in 29 (47%) of these 62 patients (16 [44%] with idiopathic and 13 [50%] with autoimmune disease). Mean (SD) time to complete response was 5.2 (5.2) months. Dapsone therapy was tapered in 21 patients after a mean (SD) of 2.4 (2.2) months and discontinued in 18. Ten patients experienced remission with no subsequent flares, even after dapsone therapy was discontinued with follow-up of 0.3 to 10.0 months. Sixteen patients experienced mild adverse effects. Two serious adverse effects were reported. Conclusions and Relevance: Results of this study suggest that dapsone is a useful and well-tolerated second-line therapy for patients with CSU in whom antihistamines and other first-line agents have failed.
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Enfermedades Autoinmunes/tratamiento farmacológico , Dapsona/administración & dosificación , Inducción de Remisión/métodos , Urticaria/tratamiento farmacológico , Administración Oral , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Enfermedad Crónica , Relación Dosis-Respuesta a Droga , Femenino , Estudios de Seguimiento , Antagonistas de los Receptores Histamínicos H1/administración & dosificación , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
Background: While most of the attention regarding skin pigmentation has focused on the effects of ultraviolet radiation, the cutaneous effects of visible light (400 to 700nm) are rarely reported. OBJECTIVE: The purpose of this study was to investigate the cutaneous pigmentary response to pure visible light irradiation, examine the difference in response to different sources of visible light irradiation, and determine a minimal pigmentary dose of visible light irradiation in melanocompetent subjects with Fitzpatrick skin type III - VI. METHODS: The study was designed as a single arm, non-blinded, split-side dual intervention study in which subjects underwent visible light irradiation using LED and halogen incandescent light sources delivered at a fluence of 0.14 Watts/cm2 with incremental dose progression from 20 J/cm2 to 320 J/cm2. Pigmentation was assessed by clinical examination, cross-polarized digital photography, and analytic colorimetry. RESULTS: Immediate, dose-responsive pigment darkening was seen with LED light exposure in 80% of subjects, beginning at 60 Joules. No pigmentary changes were seen with halogen incandescent light exposure at any dose in any subject. CONCLUSION: This study is the first to report a distinct difference in cutaneous pigmentary response to different sources of visible light, and the first to demonstrate cutaneous pigment darkening from visible LED light exposure. Our findings raise the concern that our increasing daily artificial light surroundings may have clandestine effects on skin biology.
J Drugs Dermatol. 2017;16(11):1105-1110.
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Pigmentación de la Piel/efectos de la radiación , Piel/efectos de la radiación , Adulto , Nalgas , Relación Dosis-Respuesta en la Radiación , Femenino , Humanos , Incandescencia , Luz , Masculino , Rayos Ultravioleta , Adulto JovenRESUMEN
BACKGROUND: While most of the attention regarding skin pigmentation has focused on the effects on ultraviolet radiation, the cutaneous effects of visible light (400 to 700nm) are rarely reported. In this report, we describe a case of painful erythema and induration that resulted from direct irradiation of UV-naïve skin with visible LED light in a patient with Fitzpatrick type II skin.
METHODS AND RESULTS: A 24-year-old healthy woman with Fitzpatrick type II skin presented to our department to participate in a clinical study. As part of the study, the subject underwent visible light irradiation with an LED and halogen incandescent visible light source. After 5 minutes of exposure, the patient complained of appreciable pain at the LED exposed site. Evaluation demonstrated erythema and mild induration. There were no subjective or objective findings at the halogen incandescent irradiated site, which received equivalent fluence (0.55 Watts / cm2). The study was halted as the subject was unable to tolerate the full duration of visible light irradiation.
CONCLUSION: This case illustrates the importance of recognizing the effects of visible light on skin. While the vast majority of investigational research has focused on ultraviolet light, the effects of visible light have been largely overlooked and must be taken into consideration, in all Fitzpatrick skin types.
J Drugs Dermatol. 2017;16(4):388-392.
.Asunto(s)
Eritema/etiología , Luz/efectos adversos , Pigmentación de la Piel/efectos de la radiación , Piel/efectos de la radiación , Adulto , Nalgas/efectos de la radiación , Relación Dosis-Respuesta en la Radiación , Femenino , Humanos , Incandescencia/efectos adversosRESUMEN
We present a 38-year-old woman with local heat urticaria confirmed by heat provocation testing. Heat urticaria is a rare form of physical urticaria that istriggered by exposure to a heat source, such as hot water or sunlight. Although it is commonly localized and immediate, generalized and delayed onset forms exist. Treatment options include antihistamines and heat desensitization. A brisk, mechanical stroke elicited a linear wheal. Five minutes after exposure to hot water, she developed well-demarcated,erythematous blanching wheals that covered the distal forearm and entire hand.
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Calor/efectos adversos , Urticaria/etiología , Adulto , Femenino , Humanos , Urticaria/diagnósticoRESUMEN
Aquagenic papulotranslucent acrokeratoderma isa rare condition with the development of white-totransluscentpapules and plaques after exposureto water. While the first report was described asan autosomal dominant hereditary condition,there have since been acquired cases reported inassociation with cystic fibrosis, with prior exposureto a drug, or as an idiopathic condition. We presenta 24-year-old man with acquired aquagenicpapulotranslucent acrokeratoderma that has beenpresent since infancy, without a family history,without prior drug exposure, and without anypersonal or family history of cystic fibrosis. Thus fartreatment with urea cream, calipotriene ointment,vitamin E cream, and clobetasol ointment hasbeen ineffective. Our patient will be treated withbotulinum toxin.
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Dermatosis del Pie/diagnóstico , Dermatosis de la Mano/diagnóstico , Queratosis/diagnóstico , Agua , Humanos , Masculino , Adulto JovenRESUMEN
The association between multiple pilomatricomasand the autosomal dominant neurodegenerativedisorder myotonic dystrophy has been described inthe literature. Although the mechanism is unknown,it is hypothesized that the dystrophia myotonicaprotein kinase mutation in myotonic dystrophyaffects intracellular calcium levels, which alterproliferation and terminal differentiation that leads tocells that are observed in pilomatricomas. We presenta patient with multiple, symptomatic pilomatricomasand myotonic dystrophy, with a strong family historyof both of these rare disorders.
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Enfermedades del Cabello/diagnóstico , Distrofia Miotónica/complicaciones , Neoplasias Primarias Múltiples/diagnóstico , Pilomatrixoma/diagnóstico , Neoplasias Cutáneas/diagnóstico , Adulto , Dorso , Antebrazo , Enfermedades del Cabello/complicaciones , Enfermedades del Cabello/patología , Humanos , Masculino , Neoplasias Primarias Múltiples/complicaciones , Neoplasias Primarias Múltiples/patología , Pilomatrixoma/complicaciones , Pilomatrixoma/patología , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patologíaRESUMEN
We report a 68-year-old woman with chroniclymphocytic leukemia, who developed numerous,pruritic, edematous, and vesicobullous skin lesionsof the face and extremities over the course of severalmonths. The diagnosis of eosinophilic dermatosis ofhematologic malignancy (EDHM) was made basedon the clinical history and histopathologic features.Owing to the possible link between EDHM and amore aggressive underlying CLL, she was startedagain on chemotherapy. This case serves as areminder that, although the precise pathogenesis ofEDHM remains unclear, the paraneoplastic disorderis the result of immune dysregulation. Patientswho develop EDHM should undergo prompthematologic/oncologic evaluation.
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Eosinofilia/diagnóstico , Leucemia Linfocítica Crónica de Células B/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Enfermedades de la Piel/diagnóstico , Anciano , Eosinofilia/complicaciones , Eosinofilia/patología , Dermatosis Facial/complicaciones , Dermatosis Facial/diagnóstico , Dermatosis Facial/patología , Femenino , Humanos , Dermatosis de la Pierna/complicaciones , Dermatosis de la Pierna/diagnóstico , Dermatosis de la Pierna/patología , Síndromes Paraneoplásicos/complicaciones , Síndromes Paraneoplásicos/patología , Enfermedades de la Piel/complicaciones , Enfermedades de la Piel/patologíaRESUMEN
OBJECTIVE: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. DESIGN: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm(2) (purpura threshold fluences), a pulse duration of 450µsec, and a spot size of 5 or 10mm. SETTING: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. PARTICIPANTS: Ten adult subjects who presented with flushing with dysesthesia. MEASUREMENTS: PARTICIPANTS subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). RESULTS: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm(2). Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. OBJECTIVEly, subjects demonstrated at least a 62.5-percent reduction in erythema. CONCLUSION: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia.
RESUMEN
Cutaneous flushing and facial erythema are common dermatologic conditions that elicit a wide differential diagnosis that includes rosacea, seborrheic dermatitis, photodermatitis, connective-tissue diseases, carcinoid syndrome, and mastocytosis. Herein we present an usual case of a mask-like rosacea-PIPA overlap that occurred in a patient with prior history of rectal carcinoid tumor and a negative systemic evaluation.
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Dermatosis Facial/diagnóstico , Trastornos por Fotosensibilidad/diagnóstico , Rosácea/diagnóstico , Piel/patología , Biopsia , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Piel/efectos de la radiaciónRESUMEN
Chronic actinic dermatitis (CAD) is a photosensitivity disorder that is characterized by a persistent eczematous eruption in sun-exposed sites. The hallmark of CAD is a reduced minimal erythema dose (MED) to ultraviolet B (UVB), ultraviolet A (UVA), and/or to visible light, which makes phototesting the essential diagnostic investigation. The uncommon subgroup of patients with atopic dermatitis (AD) that are affected by CAD has primarily been described in young patients in the United Kingdom. We present an atopic adult women with CAD who was diagnosed years after symptoms began. We believe it is important that dermatologists perform phototests on AD patients with features of a photoaggravated dermatitis in order to avoid delay in diagnosis of a true photosensitivity condition and provide appropriate management.
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Dermatitis Atópica/complicaciones , Trastornos por Fotosensibilidad/diagnóstico , Piel/patología , Rayos Ultravioleta/efectos adversos , Biopsia , Dermatitis Atópica/diagnóstico , Femenino , Humanos , Persona de Mediana Edad , Pruebas del Parche , Trastornos por Fotosensibilidad/complicaciones , Piel/efectos de la radiaciónRESUMEN
BACKGROUND: Chronic actinic dermatitis is a photosensitivity disorder with scant epidemiologic data. Case series in Europe have previously shown that improvement or resolution of chronic actinic dermatitis occurs over time in most patients. However, the natural history of chronic actinic dermatitis in patients in the United States has not been studied. OBJECTIVE: To study the natural history of chronic actinic dermatitis in patients in the United States. METHODS: We performed a retrospective chart review and telephone questionnaire after a 3- to 19-year follow-up period. RESULTS: Of 20 patients with chronic actinic dermatitis, 7 patients (35%) experienced resolution and an additional 11 patients (55%) experienced improvement of their photosensitivity to sunlight during the follow-up period. The proportion of patients experiencing improvement or resolution of their chronic actinic dermatitis increased at 5, 10, and 15 years after diagnosis. CONCLUSIONS: Our study demonstrates that abnormal photosensitivity to sunlight in chronic actinic dermatitis improves or resolves over time in most patients in New York. The rates of improvement or resolution in our patients in New York are similar to the rates in case series in Europe despite likely patient demographic differences.