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Carcinoma de Células Escamosas , Piel , Sulfonamidas , Humanos , Glándulas Ecrinas , Carbamatos , MetaplasiaAsunto(s)
Calcifilaxia , Calcifilaxia/complicaciones , Calcifilaxia/diagnóstico , Humanos , TiosulfatosRESUMEN
Vedolizumab is a humanized monoclonal antibody that binds to the human a4ß7 integrin and is approved for use in inflammatory bowel diseases. We describe a patient with severe, refractory erosive gingivostomatitis, which appeared a few days after the first dose of vedolizumab and resolved after discontinuation of the drug. We believe the gingivostomatitis to be a direct side effect of vedolizumab, rather than an extraintestinal manifestation of the underlying inflammatory bowel diseases. The clinicians need to be aware of this adverse event, which could be mistakenly considered as an extraintestinal manifestation of inflammatory bowel diseases.
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Anticuerpos Monoclonales Humanizados/efectos adversos , Colitis Ulcerosa/tratamiento farmacológico , Fármacos Gastrointestinales/efectos adversos , Gingivitis/inducido químicamente , Estomatitis/inducido químicamente , Adulto , Gingivitis/patología , Humanos , Masculino , Mucosa Bucal/patología , Estomatitis/patologíaAsunto(s)
Sistemas de Liberación de Medicamentos/métodos , Micosis Fungoide/tratamiento farmacológico , Fotoquimioterapia/métodos , Adulto , Anciano , Femenino , Humanos , Rayos Láser , Masculino , Persona de Mediana Edad , Micosis Fungoide/patología , Fotoquimioterapia/efectos adversos , Inducción de Remisión/métodos , Resultado del TratamientoAsunto(s)
Síndrome de Retracción de Duane/complicaciones , Neoplasias Faciales/congénito , Nevo Pigmentado/congénito , Neoplasias Cutáneas/congénito , Estrabismo/congénito , Nervio Abducens/anomalías , Neoplasias Faciales/complicaciones , Pérdida Auditiva Sensorineural/congénito , Pérdida Auditiva Sensorineural/etiología , Humanos , Lactante , Nevo Pigmentado/complicaciones , Neoplasias Cutáneas/complicaciones , Estrabismo/etiologíaRESUMEN
BACKGROUND: Genetics discoveries have allowed for a better understanding of capillary malformations (CMs) associated with overgrowth syndrome. However, molecular analyses are still not easy to perform or interpret. Other analytical methods are needed. OBJECTIVES: To identify clinical and haemodynamic factors associated with leg length discrepancy (LLD) in children with CMs of the lower limbs. METHODS: Data were obtained from the multicentre French national cohort CONAPE (COhorte Nationale d'enfants atteints d'Angiome Plan de membrE inférieur), from children aged 2-12 years old with CMs of the lower limbs. Clinical characteristics were prospectively collected. Haemodynamic factors were measured by an sonographer who calculated the arterial blood flow (ABF) in both lower limbs. An ABF difference ≥ 50% between the two lower limbs was considered relevant. LLD ≥ 2% was determined by the same radiologist on centralized radiographs. RESULTS: We analysed data at baseline for 96 children. The mean ± SD age was 5·6 ± 3·1 years; 49 (51%) were male; and 14 (15%) showed LLD. In total, 32 patients (33%) had venous anomalies, 13 (14%) lymphatic anomalies and in one child a diagnosis of Parkes Weber syndrome was made. Only an increased circumference above the knee was more frequent with than without LLD (43% vs. 13%, P = 0·02). In all, 10/79 patients (13%) showed a difference in ABF ≥ 50%: four had LLD. The frequency of differences in ABF ≥ 50% was greater with than without LLD [33% (n = 4/12) vs. 9% (n = 6/67), P = 0·04]. CONCLUSIONS: ABF measured by Duplex ultrasonography is a simple, low-cost and noninvasive complementary examination for help in detecting LLD, with a difference of ≥ 50% possibly associated.
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Velocidad del Flujo Sanguíneo/fisiología , Capilares/anomalías , Diferencia de Longitud de las Piernas/fisiopatología , Pierna/irrigación sanguínea , Malformaciones Vasculares/fisiopatología , Capilares/fisiopatología , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Masculino , Estudios Prospectivos , Factores de Riesgo , Ultrasonografía Doppler DúplexRESUMEN
Melanoma is an aggressive tumor with advanced disease characterized by widespread metastatic lesions and the tumor has traditionally been resistant to most forms of treatment. Indeed, metastatic melanoma has a very poor prognosis with a median survival time of 8-9 months and an estimated 3-year survival rate of less than 15%. Recent advances in our understanding of the genetic profile of melanoma cells and the molecular factors that drive malignant transformation have resulted in the identification of numerous new therapeutic targets. KIT is an established therapeutic target in cancers with activating mutations of KIT, such as gastrointestinal stromal tumors (GIST), and considerable efficacy has been achieved with various small molecule inhibitors of KIT including imatinib mesylate. Nilotinib is an inhibitor of ligand-induced PDGFRα and PDFGRß kinase activity and autophosphorylation of constitutively activated KIT harboring exon 13 or exon 11 mutations (IC50 values of 0.2 and 0.027 µmol/L, respectively), with efficacy comparable to that of imatinib. We report a case of non-kit mutated metastatic vaginal melanoma showing impressive response to nilotinib.
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ADN de Neoplasias/genética , Melanoma/secundario , Mutación , Proteínas Proto-Oncogénicas c-kit/genética , Pirimidinas/uso terapéutico , Neoplasias Cutáneas/secundario , Análisis Mutacional de ADN , Femenino , Humanos , Melanoma/genética , Melanoma/metabolismo , Persona de Mediana Edad , Metástasis de la Neoplasia , Proteínas Tirosina Quinasas , Proteínas Proto-Oncogénicas c-kit/metabolismo , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/metabolismoAsunto(s)
Enfermedad de Darier/complicaciones , Adulto , Niño , Preescolar , Femenino , Humanos , Hipopigmentación/etiología , Lactante , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
Variations in the clinical and histological presentation of cutaneous T-cell lymphoma (CTCL) can hamper diagnosis. We report two cases of a novel presentation of CTCL characterized by an aberrant immunophenotype with complete loss of pan T-cell antigens including T-cell receptor ß chain and showing the clinical and histopathological appearance of erythrodermic and plaque-stage mycosis fungoides.
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Linfoma Cutáneo de Células T/diagnóstico , Micosis Fungoide/diagnóstico , Neoplasias Cutáneas/diagnóstico , Anciano , Antígenos de Diferenciación de Linfocitos T/inmunología , Diagnóstico Diferencial , Humanos , Linfoma Cutáneo de Células T/inmunología , Masculino , Persona de Mediana Edad , Micosis Fungoide/inmunología , Fenotipo , Receptores de Antígenos de Linfocitos T alfa-beta/deficiencia , Neoplasias Cutáneas/inmunologíaRESUMEN
Calciphylaxis is a rare cause of skin ulcerations and necrosis in patients with both normal renal and parathyroid function. Although calciphylaxis appears to be on the increase, treatments are mainly empirical, especially for wound care. The lesions in calciphylaxis are typically very painful and carry a high risk of infection, with sepsis being the leading cause of death in this serious disease. We report two cases of nonuraemic calciphylaxis, which responded to treatment with pamidronate and wound management by negative pressure system.
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Antiinflamatorios/uso terapéutico , Calcifilaxia/terapia , Difosfonatos/uso terapéutico , Terapia de Presión Negativa para Heridas/métodos , Anciano , Terapia Combinada/métodos , Femenino , Humanos , Persona de Mediana Edad , Obesidad Mórbida/complicaciones , Pamidronato , Resultado del TratamientoRESUMEN
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta, characterized by an acute onset of ulceronecrotic papules, rapidly coalescing into large ulcers with necrotic crusts, associated with high fever and severe systemic symptoms. We report a case of a 65-year-old woman with a resistant form of FUMHD successfully treated with a tumor necrosis factor-α (TNFα) inhibitor (infliximab). After 1 year of treatment, because of the recurrence of lesions and -occurrence of severe sepsis, we decided to change the therapeutic procedure by introducing intravenous immunoglobulin witch induced a spectacular improvement. Only few cases of FUMHD treated with intravenous immunoglobulin have been reported to date. In our case, we describe the first utilization of TNFα inhibitors in the treatment of FUMHD: TNFα inhibitors may be useful, particularly in resistant cases. Further reports are required to confirm this potential therapeutic option.
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Anticuerpos Monoclonales/uso terapéutico , Fármacos Dermatológicos/uso terapéutico , Herpes Simple/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Pitiriasis Liquenoide/tratamiento farmacológico , Anciano , Quimioterapia Combinada , Femenino , Humanos , Infliximab , Factores de Tiempo , Resultado del Tratamiento , Factor de Necrosis Tumoral alfa/antagonistas & inhibidoresAsunto(s)
Elefantiasis/tratamiento farmacológico , Dermatosis de la Mano/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Mixedema/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Masculino , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
BACKGROUND: Apomorphine is a specific dopaminergic agonist used in the treatment of severe fluctuations of Parkinson's disease, particularly in patients on L-dopa. The drug is usually given subcutaneously, either as several daily injections or via a continuous subcutaneous delivery system. We describe two cases of localized cutaneous necrosis at the points of subcutaneous apomorphine injection. OBSERVATIONS: Two male patients presenting Parkinson's disease were treated by subcutaneous injection of apomorphine. One month later, asymptomatic necrotic lesions measuring from 2 to 5 mm appeared at the injection sites. Complete blood count, standard and advanced coagulation studies and screening tests were normal. One patient had taken acetylsalicylic acid. A skin biopsy showed normal epidermis, oedema of the papillary dermis with perivascular lymphocytic infiltrates, reticular dermal infiltrate with neutrophils, and necrosis of the reticular dermis and hypodermis in one patient, and in the other, necrosis in the epidermis, dermis, hypodermis and skin appendages, with dermal leucocytoclastic vasculitis and cytosteatonecrosis. Due to the severity of necrosis, apomorphine was stopped, resulting in improvement of skin lesions in one patient. In the second, due to the localized nature of the lesions and the improvement in the patient's quality of life since the introduction of apomorphine, the drug was continued, resulting in the appearance of new lesions, which continued to be limited to the injection sites. COMMENTS: To our knowledge, this is the first description of biopsy-proven apomorphine-induced localized skin necrosis. Reported cutaneous side effects of the drug include pruritic subcutaneous nodules corresponding to panniculitis with large numbers of eosinophils, allergic contact dermatitis, pigmented nodules resulting from oxidation of apomorphine, and nonspecific rashes. Cutaneous necrosis at injection sites could arise through various mechanisms: localized vasoconstriction ("dopamine necrosis"), direct toxicity of the injected drug, local manifestations of pre-existent coagulation disorders, immunological mechanisms or poor administration technique involving intravascular injection. The specific pharmacodynamic properties of apomorphine rule out vasomotor phenomena in the aetiology of such necrosis. Screening tests for thrombophilia were negative in the first patient. Although the underlying mechanism of this form of necrosis remains unknown, an immunological mechanism of the immune complex type could be considered aetiologically relevant on histological grounds due to the presence of vasculitis in one of the two patients.
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Antiparkinsonianos/efectos adversos , Apomorfina/efectos adversos , Inyecciones Subcutáneas/efectos adversos , Necrosis/inducido químicamente , Piel/patología , Anciano , Anciano de 80 o más Años , Antiparkinsonianos/administración & dosificación , Apomorfina/administración & dosificación , Humanos , MasculinoAsunto(s)
Ceguera/etiología , Celulitis (Flemón)/complicaciones , Dermatosis Facial/complicaciones , Oftalmoplejía/etiología , Infecciones Estreptocócicas/complicaciones , Anciano , Antibacterianos/uso terapéutico , Celulitis (Flemón)/tratamiento farmacológico , Dermatosis Facial/tratamiento farmacológico , Dermatosis Facial/microbiología , Humanos , Masculino , Infecciones Estreptocócicas/tratamiento farmacológico , Streptococcus pyogenes/aislamiento & purificación , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Eosinophilic dermatosis of hematologic disease (EDH) or insect bite-like reaction is a pruritic dermatitis described mostly in patients with chronic lymphocytic leukaemia (CLL). We describe six patients with the disorder in association with CLL and other blood dyscrasias. PATIENTS AND METHODS: We reviewed the medical records of patients with EDH seen between 2004 and 2009 in our department and re-examined histological slides. RESULTS: Mean age at dermatosis onset was 75.6 years and the sex ratio was 1. There were three CLL, two mantle-cell lymphomas and one MALT-type lymphoma. The dermatitis was quite polymorphic, with erythematous papules, wheals and plaques. The initial skin lesions appeared at the same time as or after the diagnosis of haematological neoplasm. Their reappearance heralded relapse of the blood disease in three cases. Histologically, all lesions had a dense dermal infiltrate of small, mostly CD4+ T-cells, with numerous eosinophils. In three patients, there was marked folliculotropism, resembling folliculotropic T-cell lymphoma. In most cases, EDH disappeared after appropriate chemotherapy for the blood disorder. DISCUSSION: Our cases show that the clinical expression of EDH is quite polymorphic. Its appearance may precede relapse of or may indicate prompt search screening for blood dyscrasia. The most efficient treatment of this dermatosis appears to be specific chemotherapy for the blood dyscrasia. There is reason to believe that a population of T-helper 2 (Th2) lymphocytes, reactive to malignant B-cells, induces tissue eosinophilia, mainly through production of interleukin (IL)-5, among other cytokines. Eosinophils appear to be the main effector cells.
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Eosinofilia/complicaciones , Leucemia Linfocítica Crónica de Células B/complicaciones , Linfoma/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Enfermedades Cutáneas Eccematosas/complicaciones , Anciano , Anciano de 80 o más Años , Eosinofilia/diagnóstico , Femenino , Humanos , Masculino , Recurrencia Local de Neoplasia/diagnóstico , Síndromes Paraneoplásicos/complicaciones , Estudios Retrospectivos , Enfermedades Cutáneas Eccematosas/diagnósticoRESUMEN
We report two cases of cutaneous granuloma induced by anti-TNF-alpha therapy: a 47-year-old man suffering from psoriatic arthritis treated with infliximab and a 56-year-old woman treated with adalimumab for polyarticular juvenile rheumatoid arthritis. The biospies confirmed the diagnosis of a 'sarcoidosis-like' reaction. No systemic involvement was observed. Such cases of noninfectious granulomatous diseases occurring during anti-TNF-alpha therapy are becoming increasingly frequent.
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Anticuerpos Monoclonales/efectos adversos , Artritis Juvenil/tratamiento farmacológico , Artritis Psoriásica/tratamiento farmacológico , Inmunoglobulina G/efectos adversos , Sarcoidosis/etiología , Enfermedades de la Piel/etiología , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Adalimumab , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales Humanizados , Etanercept , Femenino , Humanos , Inmunoglobulina G/uso terapéutico , Infliximab , Masculino , Persona de Mediana Edad , Receptores del Factor de Necrosis Tumoral/uso terapéutico , Sarcoidosis/diagnóstico , Sarcoidosis/patología , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/patologíaRESUMEN
BACKGROUND: Heparin-induced bullous hemorrhagic dermatosis is a rare, recently described side-effect of subcutaneous heparin injection. We describe a patient simultaneously presenting distant haemorrhagic bullae and eczematous reaction at the low molecular-weight heparin (LMWH) injection sites. PATIENTS AND METHODS: Subcutaneous enoxaparin sodium was initiated in a 51-year-old patient and was replaced a few days later by tinzaparin sodium. Forty-eight hours later, annular, erythematous and vesicular plaques appeared at the injection sites (thighs). Small hemorrhagic bullae were noted on the abdominal skin at the same time. Skin biopsies revealed respectively eczematous dermatitis and an intraepidermal blister filled with red blood cells. Direct immunofluorescence was negative. Standard laboratory investigations and coagulation studies were unremarkable. Skin lesions disappeared ten days after discontinuation of LMWH. Patch tests and intradermal tests were negative. DISCUSSION: The case described herein shares the stereotypical clinical picture previously reported, namely small, multiple, haemorrhagic bullae on normal skin, appearing at remote sites five to 21 days after the start of subcutaneous heparin treatment. Despite the absence of clear management guidelines, it is obviously tempting to stop the heparin if there are too many bullae for fear of more clinically significant and dangerous mucous membrane lesions. The underlying physiopathological mechanism is poorly understood; no coagulation abnormalities were recorded. In addition, our patient presented an eczematous reaction at the injection sites, raising the possibility of a type IV hypersensitivity reaction. The association of these two cutaneous side effects of heparin is perhaps not purely coincidental.
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Erupciones por Medicamentos/etiología , Erupciones por Medicamentos/inmunología , Hemorragia/inducido químicamente , Hemorragia/inmunología , Heparina de Bajo-Peso-Molecular/efectos adversos , Enfermedades Cutáneas Vesiculoampollosas/inducido químicamente , Enfermedades Cutáneas Vesiculoampollosas/inmunología , Humanos , Inmunidad Celular , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Bortezomib (Velcade) is a proteasome inhibitor used in the treatment of myeloma and other blood dyscrasias. We report the cases of two patients who developed a peculiar toxic rash suggestive of Sweet's syndrome while receiving bortezomib; one patient also presented giant mucous membrane ulcerations. PATIENTS AND METHODS: Case 1: bortezomib treatment was started in a 62-year-old man for mantle cell lymphoma. Ten days after the first treatment cycle, giant, painful oral ulcerations were noted but they resolved spontaneously. One week after the second cycle, further oral ulceration appeared, this time with a papulonodular skin rash. Histology showed neutrophilic dermal infiltrates in the skin with predominantly lymphocytic inflammation of the oral mucosa. Bortezomib was stopped and all lesions resolved with colchicine treatment. Case 2: a 46-year-old woman was receiving bortezomib treatment for plasma cell leukemia. A febrile skin rash appeared two days after the first treatment cycle but resolved spontaneously. After the first bortezomib injection during the next cycle, painful papules and nodules appeared on the trunk. The skin biopsy results were consistent with Sweet's syndrome. The lesions disappeared spontaneously. Dexamethasone was administered concomitantly with bortezomib in the ensuing cycles and there was no relapse of the skin lesions. DISCUSSION: Bortezomib-induced skin lesions are common and usually do not justify treatment withdrawal. Published observations of bortezomib-induced eruption occasionally show clinical and histological features of Sweet's syndrome, but there has been no mention of oral mucosal ulcerations. In our cases, these could be related to bortezomib-induced neutrophilic dermatosis.
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Antineoplásicos/efectos adversos , Ácidos Borónicos/efectos adversos , Pirazinas/efectos adversos , Síndrome de Sweet/inducido químicamente , Biopsia , Bortezomib , Colchicina/uso terapéutico , Dexametasona/uso terapéutico , Femenino , Humanos , Leucemia de Células Plasmáticas/etiología , Linfoma de Células del Manto/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Úlcera Cutánea/inducido químicamente , Úlcera Cutánea/patología , Síndrome de Sweet/tratamiento farmacológico , Síndrome de Sweet/patología , Resultado del TratamientoRESUMEN
Sweet's syndrome (acute febrile neutrophilic dermatosis, SS) may be considered a reactional dermatosis with numerous associated diseases. We describe a rare association between SS and acute sarcoidosis in a 55-year-old woman. The atypical feature of positive tuberculin reaction is discussed. In this association, diagnosis may be challenging with coexistent skin lesions of both diseases. Acute sarcoidosis should be considered when SS is accompanied with mediastinal lymphadenopathy, uveitis or erythema nodosum. Deep-seated nodular lesions in the context of SS are not always 'deep' SS lesions.