RESUMEN
BACKGROUND: Chronic photosensitivity dermatitis (CPD) (also named actinic reticuloid) is an unusual disease classically referred often in elderly men. Affected patients have severely itchy, thickened dry skin in areas exposed to the sun throughout the years. METHOD: A Caucasian female patient who worked most of her life outside who had "chronic dermatitis" in her neck started planting chrysanthemum in her garden on a sunny day. Later, she presented edema, erythema, papules, and a few vesicles in her neck with severe pruritus. STUDIES: A skin biopsy revealed the diagnosis of CPD, along with positive testing for ultraviolet B (UVB), minimal erythema doses (MED) for UVB (MEDB) UVA (MEDA) and PhotoPath. RESULTS: Direct immunofluorescence (DIF) stains using anti-human antibodies against fibrinogen, albumin, IgG, IgM, lambda, kappa, and C3c and C1q were positive at the base membrane area of the dermal epidermal junction, in the papillary dermis, as well as the neurovascular bundles in all the dermis and the extracellular matrix, especially those under the blisters. CONCLUSION: With this case, we suggest not forgetting the importance of using DIF in reactivated CPD cases in addition to the photo patch testing.
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Dermatitis Fotoalérgica , Humanos , Femenino , Enfermedad Crónica , Dermatitis Fotoalérgica/patología , Dermatitis Fotoalérgica/etiología , Persona de Mediana Edad , Trastornos por Fotosensibilidad/patología , Técnica del Anticuerpo Fluorescente Directa , Rayos Ultravioleta/efectos adversosAsunto(s)
Anticuerpos Monoclonales Humanizados , Trastornos por Fotosensibilidad , Inducción de Remisión , Anciano , Humanos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Enfermedad Crónica , Fármacos Dermatológicos/uso terapéutico , Trastornos por Fotosensibilidad/tratamiento farmacológico , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del TratamientoAsunto(s)
Aminopiridinas , Benzamidas , Ciclopropanos , Humanos , Aminopiridinas/efectos adversos , Aminopiridinas/administración & dosificación , Ciclopropanos/administración & dosificación , Ciclopropanos/efectos adversos , Ciclopropanos/uso terapéutico , Benzamidas/administración & dosificación , Benzamidas/efectos adversos , Femenino , Inhibidores de Fosfodiesterasa 4/administración & dosificación , Inhibidores de Fosfodiesterasa 4/efectos adversos , Crema para la Piel/administración & dosificación , Crema para la Piel/efectos adversos , Trastornos por Fotosensibilidad/tratamiento farmacológico , Trastornos por Fotosensibilidad/diagnóstico , Trastornos por Fotosensibilidad/inducido químicamente , Masculino , Persona de Mediana EdadAsunto(s)
Humanos , Trastornos por Fotosensibilidad , Albinismo , Vías Visuales , Rehabilitación , Atrofia ÓpticaRESUMEN
BACKGROUND/PURPOSE: Amidst the emergence of new therapeutic options, traditional therapeutic plasmapheresis (TPE) used in diseases involving a toxic substance in the plasma, remains a viable alternative for cases of recalcitrant solar urticaria (SU). We emphasize the importance of documenting successful experience with repeated plasmapheresis to increase awareness amongst physicians and dermatologists regarding this effective treatment option. MATERIAL AND METHOD: We reported a case of recalcitrant SU that had not responded to a combination of H1-antihistamines, immunosuppressants, omalizumab and intravenous immunoglobulin. We introduced serial TPE, which involved two consecutive days of procedures for each course was introduced. We detailed the regimen and highlighted the clinical and objective benefits observed with multiple treatments. Additionally, we compared this to other plasmapheresis regimens and their treatment responses previously reported for solar urticaria. RESULTS: Our patient underwent serial TPE, totaling 42 procedures over five years. Following the last TPE session, phototesting showed a sustained prolongation of minimal urticating doses (MUDS), which exceeded the maximum tested doses across nearly all ultraviolet (UV) and visible light ranges, with the exception of the two short ultraviolet B (UVB) wavelengths. MUDs increased to 25 from 6 mj/cm2 at 307.5± 5nm, and to 500 from 15 mj/cm2 at 320 ± 10nm, before the initial TPE. In our review, we included five articles covering eight SU patients who received TPE. Of these, the five patients with positive intradermal tests responded particularly well immediately after treatment. However, the condition relapsed within two weeks in one patient and within two months in another. In contrast, the other three patients with negative intradermal tests, showed no significant benefits from the treatment. No serious side effects from TPE were reported amongst the patients. CONCLUSIONS: This review underscores the efficacy of serial plasmapheresis procedures in treating refractory cases of SU, high3lighting the robust results observed.
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Plasmaféresis , Urticaria , Humanos , Urticaria/terapia , Resultado del Tratamiento , Femenino , Luz Solar/efectos adversos , Masculino , Trastornos por Fotosensibilidad/terapia , Adulto , Persona de Mediana Edad , Urticaria SolarRESUMEN
Solar urticaria is a rare idiopathic photodermatosis. According to the current knowledge its pathogenesis is most likely based on an allergic type I reaction to an autoantigen activated by ultraviolet (UV) radiation or visible light. As many of the patients suffer from severe forms of the disease, it may therefore severely impair the quality of life of those affected. In contrast, polymorphous light eruption is a very common disease, which, according to the current data, can be interpreted as a type IV allergic reaction to a photoallergen induced by UV radiation. As the skin lesions heal despite continued sun exposure, the patients' quality of life is generally not significantly impaired. These two clinically and pathogenetically very different light dermatoses have shared diagnostics by means of light provocation and an important therapeutic option (light hardening). Herein, we present an overview of the clinical picture, pathogenesis, diagnosis and available treatment options for the above-mentioned diseases.
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Trastornos por Fotosensibilidad , Urticaria , Humanos , Urticaria/etiología , Urticaria/inmunología , Urticaria/diagnóstico , Trastornos por Fotosensibilidad/diagnóstico , Trastornos por Fotosensibilidad/etiología , Trastornos por Fotosensibilidad/terapia , Trastornos por Fotosensibilidad/inmunología , Luz Solar/efectos adversos , Rayos Ultravioleta/efectos adversos , Dermatitis Fotoalérgica/diagnóstico , Dermatitis Fotoalérgica/etiología , Diagnóstico Diferencial , Urticaria SolarRESUMEN
Photosensitivity represents an increased inflammatory reaction to sunlight, which can be observed particularly in the autoimmune disease lupus erythematosus. Cutaneous lupus erythematosus (CLE) can be provoked by ultraviolet (UV) radiation and can cause both acute, nonscarring and chronic, scarring skin changes. In systemic lupus erythematosus, on the other hand, provocation by UV radiation can lead to flare or progression of systemic involvement. The etiology of lupus erythematosus is multifactorial and includes genetic, epigenetic and immunologic mechanisms. In this review, we address the effect of UV radiation on healthy skin and photosensitive skin using the example of lupus erythematosus. We describe possible mechanisms of UV-triggered immune responses that could offer therapeutic approaches. Currently, photosensitivity can only be prevented by avoiding UV exposure itself. Therefore, it is important to better understand the underlying mechanisms in order to develop strategies to counteract the deleterious effects of photosensitivity.
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Lupus Eritematoso Cutáneo , Rayos Ultravioleta , Humanos , Rayos Ultravioleta/efectos adversos , Lupus Eritematoso Cutáneo/etiología , Lupus Eritematoso Cutáneo/inmunología , Lupus Eritematoso Cutáneo/patología , Lupus Eritematoso Sistémico/etiología , Lupus Eritematoso Sistémico/inmunología , Trastornos por Fotosensibilidad/etiología , Trastornos por Fotosensibilidad/inmunología , Piel/efectos de la radiación , Piel/patología , Piel/inmunologíaAsunto(s)
Azetidinas , Purinas , Pirazoles , Sulfonamidas , Humanos , Azetidinas/uso terapéutico , Azetidinas/administración & dosificación , Pirazoles/administración & dosificación , Pirazoles/uso terapéutico , Sulfonamidas/administración & dosificación , Purinas/administración & dosificación , Purinas/uso terapéutico , Administración Oral , Trastornos por Fotosensibilidad/tratamiento farmacológico , Femenino , Resultado del Tratamiento , Masculino , Enfermedades Cutáneas GenéticasRESUMEN
The autoimmune disease lupus erythematosus (lupus) is characterized by photosensitivity, where even ambient ultraviolet radiation (UVR) exposure can lead to development of inflammatory skin lesions. We have previously shown that Langerhans cells (LCs) limit keratinocyte apoptosis and photosensitivity via a disintegrin and metalloprotease 17 (ADAM17)-mediated release of epidermal growth factor receptor (EGFR) ligands and that LC ADAM17 sheddase activity is reduced in lupus. Here, we sought to understand how the lupus skin environment contributes to LC ADAM17 dysfunction and, in the process, differentiate between effects on LC ADAM17 sheddase function, LC ADAM17 expression, and LC numbers. We show through transcriptomic analysis a shared IFN-rich environment in non-lesional skin across human lupus and three murine models: MRL/lpr, B6.Sle1yaa, and imiquimod (IMQ) mice. IFN-I inhibits LC ADAM17 sheddase activity in murine and human LCs, and IFNAR blockade in lupus model mice restores LC ADAM17 sheddase activity, all without consistent effects on LC ADAM17 protein expression or LC numbers. Anti-IFNAR-mediated LC ADAM17 sheddase function restoration is associated with reduced photosensitive responses that are dependent on EGFR signaling and LC ADAM17. Reactive oxygen species (ROS) is a known mediator of ADAM17 activity; we show that UVR-induced LC ROS production is reduced in lupus model mice, restored by anti-IFNAR, and is cytoplasmic in origin. Our findings suggest that IFN-I promotes photosensitivity at least in part by inhibiting UVR-induced LC ADAM17 sheddase function and raise the possibility that anifrolumab ameliorates lupus skin disease in part by restoring this function. This work provides insight into IFN-I-mediated disease mechanisms, LC regulation, and a potential mechanism of action for anifrolumab in lupus.
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Proteína ADAM17 , Células de Langerhans , Lupus Eritematoso Sistémico , Piel , Proteína ADAM17/metabolismo , Proteína ADAM17/genética , Animales , Humanos , Células de Langerhans/metabolismo , Ratones , Piel/metabolismo , Piel/patología , Piel/efectos de la radiación , Lupus Eritematoso Sistémico/metabolismo , Rayos Ultravioleta/efectos adversos , Femenino , Modelos Animales de Enfermedad , Trastornos por Fotosensibilidad/metabolismo , Interferones/metabolismo , Ratones Endogámicos MRL lprRESUMEN
A man in his 50s was diagnosed with solar urticaria following monochromated light testing that demonstrated exquisite photosensivity to ultraviolet (UV) A, UV B (UVB) and visible light.Treatment options for this photodermatosis are limited; UVB phototherapy is one modality that can be appropriate in some patients. This is administered at very low doses in a controlled environment to induce skin hardening.1 To self-treat his condition, the patient used a commercial sunbed on two occasions several days apart. He noted an immediate flare of solar urticaria after first use with associated dizziness. Following the second use, he felt generally unwell and was witnessed to lose consciousness and displayed jerky movements of his limbs while a passenger in a car. Investigations including a head MRI and an EEG were normal; an anoxic seizure caused by a flare of solar urticaria was later confirmed.Solar urticaria is a rare photodermatosis that is poorly understood and difficult to treat. The condition has a significant impact on the quality of life of patients. Severe cases can be associated with systemic symptoms that could be life-threatening.
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Trastornos por Fotosensibilidad , Luz Solar , Rayos Ultravioleta , Urticaria Solar , Humanos , Masculino , Persona de Mediana Edad , Trastornos por Fotosensibilidad/etiología , Luz Solar/efectos adversos , Rayos Ultravioleta/efectos adversos , Urticaria Solar/etiologíaRESUMEN
Patients presenting with a linear, erythematous, blistering eruption may experience a sudden painful sunburn that seems to get worse rather than better with time. In warm climates, exposure to the common fig tree (Ficus carica) may be the culprit. Dermatologists should recognize fig phytophotodermatitis as a possible cause and help the patient connect their symptoms with the inciting agent as well as administer proper treatment.
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Ficus , Humanos , Ficus/efectos adversos , Dermatitis Fototóxica/etiología , Dermatitis Fototóxica/diagnóstico , Trastornos por Fotosensibilidad/diagnóstico , Trastornos por Fotosensibilidad/etiología , Quemadura SolarRESUMEN
OBJECTIVES: This study investigated the clinically relevant factors for headaches in patients with systemic lupus erythematosus (SLE) using a registry from a Japanese multicenter cohort. METHODS: This cross-sectional study analysed the clinical information of patients with SLE who experienced headache episodes using the Migraine Disability Assessment (MIDAS) questionnaire. Significant findings in the comparisons between patients with headache (HA patients) and those without headache (non-HA patients) and in the comparisons depending on the grades of headache-induced disability in daily life based on the MIDAS scores were evaluated. Multivariate logistic regression analyses were performed to identify the relevant factors for headache. RESULTS: We analyzed 369 patients (median age, 45 years; female, 90.8%), including 113 HA patients who were significantly younger than non-HA patients (p < .005). HA patients had significantly higher frequencies of photosensitivity, rashes, and mucosal ulcers than non-HA patients (p < .05). Age and photosensitivity were significantly associated with headache (odds ratio (OR) 0.93, 95% confidence interval (CI) 0.95-0.99; OR 2.11, 95% CI 1.29-3.49, respectively). In the HA patients, hypocomplementemia was significantly associated with a disability of more than mild grade (OR 2.89, 95% CI 1.14-7.74), while rash was significantly observed in those presenting with moderate and severe disability. CONCLUSION: This study suggests that photosensitivity is a relevant manifestation of headache in patients with SLE. Persistent hypocomplementemia can contribute to headache-induced disability in daily life, whereas a rash may be a dominant manifestation in patients presenting with moderate/severe headache-induced disability.
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Cefalea , Lupus Eritematoso Sistémico , Sistema de Registros , Humanos , Femenino , Estudios Transversales , Persona de Mediana Edad , Masculino , Adulto , Lupus Eritematoso Sistémico/complicaciones , Japón/epidemiología , Cefalea/etiología , Cefalea/epidemiología , Encuestas y Cuestionarios , Modelos Logísticos , Evaluación de la Discapacidad , Índice de Severidad de la Enfermedad , Análisis Multivariante , Factores de Edad , Trastornos por Fotosensibilidad/epidemiología , Trastornos por Fotosensibilidad/etiología , AncianoRESUMEN
BACKGROUND: We aimed to investigate the prevalence of skin disease among patients with systemic lupus erythematosus (SLE) and determine whether LE skin disease had clinical or serologic correlates with SLE. METHODS: We reviewed records of 335 patients with SLE (seen at Mayo Clinic, Rochester, Minnesota, USA) and abstracted skin manifestations, fulfilled mucocutaneous SLE criteria, and clinical and serologic parameters. RESULTS: Of the 231 patients with skin manifestations, 57 (24.7%) had LE-specific conditions, 102 (44.2%) had LE-nonspecific conditions, and 72 (31.2%) had both. LE skin disease was associated with photosensitivity, anti-Smith antibodies, and anti-U1RNP antibodies (all P < 0.001). Patients without LE skin disease more commonly had elevated C-reactive protein levels (P = 0.01). Patients meeting 2-4 mucocutaneous American College of Rheumatology criteria less commonly had cytopenia (P = 0.004) or anti-double-stranded DNA antibodies (P = 0.004). No significant associations were observed for systemic involvement (renal, hematologic, neurologic, and arthritis) when comparing patients with or without LE skin involvement. LE skin involvement was not significantly associated with internal SLE disease flare, number of medications, or overall survival. CONCLUSIONS: LE skin disease commonly occurs in patients with SLE. The presence of LE skin disease had no mitigating impact on the severity of SLE sequelae, disease flares, number of medications, or overall survival.