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Piodermia Gangrenosa , Síndrome de Sweet , Vasculitis Leucocitoclástica Cutánea , Humanos , Piodermia Gangrenosa/complicaciones , Piodermia Gangrenosa/diagnóstico , Piodermia Gangrenosa/tratamiento farmacológico , Vasculitis Leucocitoclástica Cutánea/complicaciones , Vasculitis Leucocitoclástica Cutánea/diagnóstico , Vasculitis Leucocitoclástica Cutánea/tratamiento farmacológico , EritemaRESUMEN
Autoimmune diseases triggered by coronavirus disease 2019 (COVID-19) mRNA vaccination have been emerging. Here, we report the case of a 27-year-old Japanese man with autoimmunity-related neutrophilic dermatosis, occurring as an initial cutaneous manifestation of systemic lupus erythematosus with Sjögren syndrome after the second dose of the Pfizer/BioNTech COVID-19 vaccination. The patient presented with urticarial erythema and partially annular erythema on the trunk and extremities with severe pruritus. Histopathological analysis showed vacuolar degeneration at the dermo-epidermal junction and interstitial neutrophil infiltration. We reviewed eight patients, including the aforementioned patient, with exacerbation or new-onset of SLE after COVID-19 vaccination and found the patient had relatively mild symptoms, itchy annular erythema, and positive anti-SS-A/SS-B antibodies. COVID-19 mRNA vaccination can induce the production of type-I interferon, which plays a crucial role in the pathogenesis of SLE and may cause autoimmunity-related neutrophilic dermatosis in susceptible individuals. In the case that itchy annular erythema develops approximately 2 weeks after the vaccination, the possibility of systemic or cutaneous lupus erythematosus should be considered. For an accurate diagnosis, dermatologists should obtain a recent vaccination history and perform complete antibody profiling and skin biopsy for patients presenting with annular or erythema multiforme-like lesions.
Asunto(s)
Enfermedades Autoinmunes , COVID-19 , Dermatitis , Lupus Eritematoso Sistémico , Masculino , Humanos , Adulto , Autoinmunidad , Vacunas contra la COVID-19/efectos adversos , Eritema , Enfermedades Autoinmunes/etiología , Prurito/etiologíaRESUMEN
Kawasaki disease (KD) in adolescence and adulthood is often underrecognized, because KD predominantly affects infants and young children below the age of 5 years. We report four cases of KD in patients 16-32 years of age. The first department that the patients visited was the Department of Otolaryngology, Obstetrics, or General Internal Medicine. Since KD almost always develops as cutaneous and mucosal manifestations, dermatologists have a particularly significant role in diagnosing KD in adolescents and adults. KD should be kept in mind for febrile patients of any age group presenting with exanthem.
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Exantema , Síndrome Mucocutáneo Linfonodular , Adolescente , Adulto , Niño , Preescolar , Exantema/diagnóstico , Exantema/etiología , Fiebre/etiología , Humanos , Lactante , Síndrome Mucocutáneo Linfonodular/diagnósticoAsunto(s)
Antineoplásicos/efectos adversos , Dasatinib/efectos adversos , Erupciones por Medicamentos/etiología , Paniculitis/inducido químicamente , Anciano , Antineoplásicos/uso terapéutico , Dasatinib/uso terapéutico , Humanos , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , MasculinoRESUMEN
Background: Endogenous DNA derived from nuclei or mitochondria is released into the blood circulation as cell-free DNA (cfDNA) following cell damage or death. cfDNA is associated with various pathological conditions; however, its clinical significance in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) remains unclear. This study aimed to evaluate the clinical significance of cfDNA in AAV. Methods: We enrolled 35 patients with AAV, including 10 with eosinophilic granulomatosis with polyangiitis (EGPA), 13 with microscopic polyangiitis, and 12 with granulomatosis with polyangiitis. Serum cf-nuclear DNA (cf-nDNA) and cf-mitochondrial DNA (cf-mtDNA) levels were measured by quantitative polymerase chain reaction before and after the initiation of immunosuppressive therapy. Tissue samples from EGPA patients were examined by immunofluorescence and transmission electron microscopy. The structure of eosinophil extracellular traps (EETs) and neutrophil extracellular traps (NETs) and stability against DNase were assessed in vitro. Platelet adhesion of EETs were also assessed. Results: Serum cf-nDNA and cf-mtDNA levels were significantly higher in AAV than in healthy controls, with the highest levels in EGPA; however, serum DNase activities were comparable among all groups. cf-nDNA and cf-mtDNA decreased after treatment and were associated with disease activity only in EGPA. Blood eosinophil count and plasma D-dimer levels were significantly correlated with cf-nDNA in EGPA and cf-mtDNA. EGPA tissue samples showed lytic eosinophils and EETs in small-vessel thrombi. The structure of EETs showed bolder net-like chromatin threads in vitro and EETs showed greater stability against DNase than NETs. EETs provided a scaffold for platelet adhesion. Conclusion: cfDNA was increased in EGPA, associated with disease activity. The presence of DNase-resistant EETs in small-vessel thrombi might contribute to higher concentration of cfDNA and the occurrence of immunothrombosis in EGPA.
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Ácidos Nucleicos Libres de Células , Eosinófilos/inmunología , Eosinófilos/metabolismo , Eosinófilos/patología , Granulomatosis con Poliangitis/etiología , Granulomatosis con Poliangitis/metabolismo , Tromboinflamación , Anciano , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Biomarcadores , Plaquetas/inmunología , Plaquetas/metabolismo , Plaquetas/patología , Plaquetas/ultraestructura , Diagnóstico Diferencial , Susceptibilidad a Enfermedades/inmunología , Trampas Extracelulares/inmunología , Trampas Extracelulares/metabolismo , Femenino , Productos de Degradación de Fibrina-Fibrinógeno , Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/terapia , Humanos , Inmunosupresores/uso terapéutico , Pruebas de Función Renal , Recuento de Leucocitos , Biopsia Líquida/métodos , Masculino , Poliangitis Microscópica/diagnóstico , Persona de Mediana Edad , Agregación Plaquetaria , Tromboinflamación/complicaciones , Tromboinflamación/diagnóstico , Tromboinflamación/etiología , Tromboinflamación/inmunologíaRESUMEN
BACKGROUND: Leiomyomas with eosinophilic intracytoplasmic inclusion bodies have been described in the urinary bladder, brain, gastrointestinal tract, uterus, and oral cavity but not in the skin. Prompted by our recent experience with a case of cutaneous angioleiomyoma with many inclusion bodies, we hypothesized that similar cases might have been previously overlooked. METHODS: We retrospectively reviewed 30 cases of angioleiomyoma and 10 cases of piloleiomyoma focusing on inclusion bodies. RESULTS: More than 18 inclusion bodies per 250 µm squared were detected in five cases of angioleiomyoma, fewer than 11 bodies in 20 cases, and none in five cases. For the case with numerous inclusion bodies throughout the specimen, special staining was needed to make a diagnosis. No inclusion bodies were found in the piloleiomyomas. CONCLUSION: Inclusion bodies are relatively common in angioleiomyomas and can occasionally be numerous. They may serve as a point of distinction from piloleiomyomas. Because the presence of multiple eosinophilic intracytoplasmic inclusions can result in a rhabdoid appearance and make diagnosis challenging, we should be aware of this feature in angioleiomyomas.
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Angiomioma , Cuerpos de Inclusión , Neoplasias Cutáneas , Adolescente , Adulto , Angiomioma/metabolismo , Angiomioma/patología , Niño , Femenino , Humanos , Cuerpos de Inclusión/metabolismo , Cuerpos de Inclusión/patología , Masculino , Estudios Retrospectivos , Neoplasias Cutáneas/metabolismo , Neoplasias Cutáneas/patologíaAsunto(s)
Benzoatos/efectos adversos , Hidrazinas/efectos adversos , Isquemia/etiología , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Pirazoles/efectos adversos , Trombosis/inducido químicamente , Anciano , Biopsia , Clopidogrel/uso terapéutico , Angiografía por Tomografía Computarizada , Tratamiento Conservador , Femenino , Humanos , Oxigenoterapia Hiperbárica , Isquemia/patología , Isquemia/terapia , Recuento de Plaquetas , Púrpura Trombocitopénica Idiopática/sangre , Piel/irrigación sanguínea , Piel/diagnóstico por imagen , Piel/patología , Trombosis/complicaciones , Trombosis/diagnóstico , Trombosis/tratamiento farmacológico , Arterias Tibiales/diagnóstico por imagen , Arterias Tibiales/patología , Dedos del Pie/irrigación sanguínea , Dedos del Pie/diagnóstico por imagen , Dedos del Pie/patología , Resultado del Tratamiento , Warfarina/uso terapéuticoRESUMEN
Azathioprine (AZA)-metabolizing enzyme gene polymorphism is strongly related to thiopurine-induced leukocytopenia, which has not been well recognized in dermatological practice. We tried to see whether NUDT15 gene polymorphism can be the most susceptible genetic factor for AZA toxicity and the gene screening is beneficial to avoid the adverse events of AZA for the treatment of skin diseases. A retrospective study was carried out on 15 adult Japanese patients who were treated with AZA. Gene polymorphism of thiopurine-metabolizing enzymes NUDT15 R139C, ITPA 94C>A, TPMT*2, TPMT*3B and TPMT*3C was analyzed. The single nucleotide polymorphisms were prospectively investigated in eight patients who were considered to have received AZA treatments. Two NUDT15 R139C homozygous patients developed agranulocytosis, severe thrombocytopenia and massive hair loss. The gene screening prior to AZA treatment identified one heterozygote of NUDT15 R139C and ITPA 94C>A, and three heterozygotes of ITPA 94C>A or TMPT*3C. Although this study was a retrospective single-center case-control observational study that enrolled a small number of patients, NUDT15 R139C homozygosity is a genetic risk of thiopurine-induced potentially fetal hematological abnormalities. To avoid serious adverse events, gene screening of thiopurine-metabolizing enzymes, at least NUDT15 R139C, should be considered prior to administration in genetically predisposed populations, such as Japanese. We highlight that massive hair loss in the early period of the initiation of AZA would be a sign of impending severe myelotoxicity.
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Alopecia/inducido químicamente , Azatioprina/efectos adversos , Inmunosupresores/efectos adversos , Leucopenia/inducido químicamente , Pirofosfatasas/genética , Enfermedades de la Piel/tratamiento farmacológico , Alopecia/genética , Pueblo Asiatico/genética , Azatioprina/metabolismo , Estudios de Casos y Controles , Femenino , Humanos , Inmunosupresores/metabolismo , Leucopenia/sangre , Leucopenia/diagnóstico , Leucopenia/genética , Masculino , Persona de Mediana Edad , Polimorfismo de Nucleótido Simple , Pirofosfatasas/metabolismo , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Enfermedades de la Piel/inmunologíaRESUMEN
Eosinophilic granulomatosis with polyangiitis (EGPA; ie, Churg-Strauss syndrome) is one of the antineutrophil cytoplasmic antibody-associated vasculitis syndromes. Although extravascular granulomatoses are a well-known histopathological feature, the diverse histopathologic spectrum of cutaneous lesions has not been described in detail. Thus, this study sought to investigate the possible correlation between the clinical features and histopathology of cutaneous lesions in EGPA cases, focusing on systemic thrombogenic conditions, such as visceral infarction and deep vein thrombosis. Fourteen cases of EGPA diagnosed at the Department of Dermatology in Asahikawa Medical University from 1977 to 2017 were clinically and histopathologically reviewed. In 6 (43%) cases, skin lesions were the initial manifestation of EGPA. Among the cutaneous lesions, purpura and erythema were the most common. Persistent proteinuria and macrohematuria were observed in only 2 myeloperoxidase-antineutrophil cytoplasmic antibody-positive cases. Systemic thrombotic symptoms, such as cerebral infarction and deep vein thrombosis, were detected in 5 (36%) cases, and, in 3 of those cases, thromboses in dermal or subcutaneous vessels were observed histopathologically. Elevation of plasma D-dimer level (>2.5 µg/mL) was significantly correlated with concomitant systemic thrombotic symptoms (P = 0.0152, Fischer exact test). The histopathological finding of thrombotic features and increased plasma D-dimer were predictive factors of EGPA accompanied with systemic thromboses, such as deep vein thromboses and cerebral infarction.
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Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/patología , Productos de Degradación de Fibrina-Fibrinógeno/análisis , Trombosis/etiología , Adulto , Anciano , Síndrome de Churg-Strauss/sangre , Femenino , Humanos , Masculino , Persona de Mediana EdadAsunto(s)
Antibacterianos/efectos adversos , Síndrome de Hipersensibilidad a Medicamentos/etiología , Neumonía por Pneumocystis/prevención & control , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/etiología , Combinación Trimetoprim y Sulfametoxazol/efectos adversos , Síndrome de Hipersensibilidad a Medicamentos/patología , Glomerulonefritis por IGA/complicaciones , Glomerulonefritis por IGA/tratamiento farmacológico , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neumonía por Pneumocystis/etiología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico por imagen , Prednisolona/uso terapéutico , Piel/patologíaAsunto(s)
Lesión Renal Aguda/etiología , Embolia/complicaciones , Polímeros/efectos adversos , Complicaciones Posoperatorias/etiología , Reemplazo de la Válvula Aórtica Transcatéter/efectos adversos , Lesión Renal Aguda/terapia , Anciano de 80 o más Años , Estenosis de la Válvula Aórtica/cirugía , Biopsia , Embolia/inducido químicamente , Embolia/patología , Humanos , Masculino , Microvasos , Complicaciones Posoperatorias/patología , Complicaciones Posoperatorias/terapia , Diálisis Renal , Piel/irrigación sanguínea , Piel/patología , Reemplazo de la Válvula Aórtica Transcatéter/instrumentaciónAsunto(s)
Acné Vulgar/genética , Proteínas Adaptadoras Transductoras de Señales/genética , Proteínas del Citoesqueleto/genética , Hidradenitis Supurativa/genética , Piodermia Gangrenosa/genética , Acné Vulgar/diagnóstico , Acné Vulgar/tratamiento farmacológico , Corticoesteroides/uso terapéutico , Adulto , Exones/genética , Femenino , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Hidradenitis Supurativa/diagnóstico , Hidradenitis Supurativa/tratamiento farmacológico , Humanos , Japón , Mutación Missense , Prednisolona/administración & dosificación , Prednisolona/uso terapéutico , Piodermia Gangrenosa/diagnóstico , Piodermia Gangrenosa/tratamiento farmacológico , Recurrencia , Síndrome , Resultado del TratamientoAsunto(s)
Crioglobulinemia/etiología , Pabellón Auricular/patología , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma no Hodgkin/complicaciones , Piel/patología , Neoplasias Gástricas/complicaciones , Anciano de 80 o más Años , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamiento farmacológico , Gangrena/tratamiento farmacológico , Gangrena/etiología , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma no Hodgkin/diagnóstico , Linfoma no Hodgkin/tratamiento farmacológico , Masculino , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/tratamiento farmacológico , Resultado del TratamientoRESUMEN
Anhidrosis/hypohidrosis are conditions presenting various level of sweating dysfunction. Among them, acquired idiopathic generalized anhidrosis (AIGA) presents inadequate decrease or loss of sweating without apparent neurological and dermatological symptoms except cholinergic urticaria. Recently, serum level of carcinoembryonic antigen (CEA), one of the most well-known tumor markers, has been proposed as a clinical marker reflecting activity of AIGA. This study was performed to verify the specificity and independence of serum CEA level from the other serum tumor markers especially related to adenocarcinoma. The expression of various tumor markers in the serum collected from three healthy control subjects, four AIGA cases, and a cholinergic urticaria (CU) case with elevation of serum CEA level and history of hyperthermia was analyzed using a membrane-based antibody array. In all AIGA and CU cases, the intensity of CEA was significantly increased (7.60-15.9 times compared with that of control), relatively well-reflecting the serum CEA level, and the mean intensity of CEA was 11.8 times higher than the control subjects (P = 0.0011). On the other hand, the ratio of carbohydrate antigen (CA)125 and CA19-9 was 1.93 and 0.23 times compared with the mean intensity of the control subjects, respectively, and there was no statistical significance. Immunohistochemistry on 10 AIGA cases showed increased expression of CEA but not CA19-9 and CA125 in the eccrine sweat glands. In conclusion, the elevation of serum CEA level was independent from the other tumor markers in hypohidrotic condition represented by AIGA.
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Antígeno Ca-125/sangre , Antígeno CA-19-9/sangre , Antígeno Carcinoembrionario/sangre , Hipohidrosis/sangre , Proteínas de la Membrana/sangre , Urticaria/sangre , Adulto , Antígeno Ca-125/metabolismo , Antígeno CA-19-9/metabolismo , Antígeno Carcinoembrionario/metabolismo , Glándulas Ecrinas/patología , Femenino , Proteínas Ligadas a GPI/sangre , Proteínas Ligadas a GPI/metabolismo , Humanos , Hipohidrosis/patología , Inmunohistoquímica , Masculino , Proteínas de la Membrana/metabolismo , Persona de Mediana Edad , Sensibilidad y Especificidad , Sudoración/fisiología , Urticaria/patologíaAsunto(s)
Anticuerpos Monoclonales Humanizados/efectos adversos , Antineoplásicos/efectos adversos , Autoinmunidad/efectos de los fármacos , Erupciones por Medicamentos/inmunología , Anciano , Humanos , Leucemia-Linfoma de Células T del Adulto , Receptores CCR4/antagonistas & inhibidores , Receptores CCR4/inmunología , Receptores CCR4/metabolismo , Linfocitos T/efectos de los fármacos , Linfocitos T/inmunología , Linfocitos T/metabolismo , Timoma/inmunología , Neoplasias del Timo/diagnóstico , Neoplasias del Timo/inmunologíaRESUMEN
OBJECTIVES/HYPOTHESIS: We aimed to test the hypothesis that chemotherapy changes the gene expression of taste receptors in the tongue to induce dysgeusia in patients with head and neck cancer. STUDY DESIGN: Prospective observation study. METHODS: We enrolled 21 patients who received chemoradiotherapy and five patients who underwent radiotherapy for head and neck cancer. The messenger RNA (mRNA) levels of the taste receptor subunits T1R1, T1R2, T1R3, and T2R5 were measured in lingual mucosa scrapings obtained with a small spatula. The perception thresholds of umami, sweet, and bitter tastes were assessed by the whole mouth gustatory test. RESULTS: In four patients with severe stomatitis induced by chemoradiotherapy, the mRNA levels of T1R1, T1R2, T1R3, and T2R5 in the lingual mucosa were significantly decreased. However, in 17 patients with mild/moderate stomatitis, the mRNA levels of T1R3 were significantly and transiently decreased, whereas those of T1R1 and T1R2 remained unchanged and those of T2R5 mRNA were significantly and transiently increased after chemotherapy. There was a significant negative correlation between the perception thresholds of umami or sweet tastes and lingual mRNA levels of T1R3 in patients with mild/moderate stomatitis after chemotherapy. Although the perception threshold of bitter taste remained unchanged, lingual mRNA levels of T2R5 were significantly increased in patients who complained of phantogeusia after chemotherapy. CONCLUSION: Chemotherapy specifically changed the gene expression of T1R3 and T2R5 in head and neck cancer patients with mild/moderate stomatitis, resulting in both dysgeusia of umami and sweet tastes as well as phantogeusia. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:E103-E109, 2016.
Asunto(s)
Quimioradioterapia/efectos adversos , Disgeusia/genética , Expresión Génica/efectos de los fármacos , Neoplasias de Cabeza y Cuello/terapia , Receptores Acoplados a Proteínas G/genética , Adulto , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Antineoplásicos/efectos adversos , Antineoplásicos/uso terapéutico , Quimioradioterapia/métodos , Estudios de Cohortes , Disgeusia/etiología , Femenino , Expresión Génica/efectos de la radiación , Neoplasias de Cabeza y Cuello/patología , Humanos , Japón , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , ARN Mensajero/genética , ARN Mensajero/metabolismo , Medición de Riesgo , Índice de Severidad de la Enfermedad , Papilas Gustativas/efectos de los fármacos , Lengua/efectos de los fármacos , Lengua/efectos de la radiaciónRESUMEN
Hypohidrosis and anhidrosis are congenital or acquired conditions which are characterized by inadequate sweating. Acquired idiopathic generalized hypohidrosis/anhidrosis (AIGA) includes idiopathic pure sudomotor failure (IPSF), which has the following distinct features: sudden onset in youth, increased serum immunoglobulin E and responds favorably to systemic corticosteroid. No clinical markers reflecting the disease severity or activity have been established. Here, we report a case of AIGA in a Japanese patient successfully treated with repeated methylprednisolone pulse therapy. In this case, serum carcinoembryonic antigen (CEA) levels increased up to 19.8 ng/mL along with aberrant CEA immunoreactivity of eccrine sweat glands. Interestingly, the serum CEA level normalized as sweating improved with repeated methylprednisolone pulse therapy. Therefore, serum CEA level may serve as a useful clinical marker of hypohidrosis or anhidrosis.