Asunto(s)
Enfermedad Injerto contra Huésped/etiología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Penfigoide Benigno de la Membrana Mucosa/inducido químicamente , Acondicionamiento Pretrasplante/efectos adversos , Humanos , Leucemia/cirugía , Masculino , Persona de Mediana Edad , Penfigoide Benigno de la Membrana Mucosa/inmunología , Trasplante HomólogoRESUMEN
We describe a case of drug rash with eosinophilia and systemic symptoms (DRESS) and graft-versus-host disease (GvHD) developing sequentially in a patient displaying reactivation of CMV. We discuss the possibility that similar pathogenic mechanisms may be involved in the development of DRESS and GvHD.
Asunto(s)
Infecciones por Citomegalovirus/inducido químicamente , Erupciones por Medicamentos/etiología , Eosinofilia/inducido químicamente , Enfermedad Injerto contra Huésped/inducido químicamente , Infecciones por Bacterias Gramnegativas , Antibacterianos/efectos adversos , Citomegalovirus/aislamiento & purificación , Erupciones por Medicamentos/patología , Eosinofilia/tratamiento farmacológico , Eosinofilia/patología , Enfermedad Injerto contra Huésped/patología , Infecciones por Bacterias Gramnegativas/tratamiento farmacológico , Infecciones por Bacterias Gramnegativas/patología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Linfocinas/inmunología , Linfoma no Hodgkin/terapia , Masculino , Persona de Mediana Edad , Activación ViralRESUMEN
Cutaneous manifestations of myelodysplastic syndromes (MDS) may predict disease progression and a poorer prognosis. We describe a patient in whom a deep neutrophilic dermatosis preceded evolution of disease from refractory anaemia to RAEB (refractory anaemia with excess blasts) and resolved completely on treating the disease with 5-azacytidine. The dermatological response was accompanied by complete bone marrow remission and trilineage haematological improvement. We suggest that 5-azacytidine should be considered in the treatment of immune mediated cutaneous manifestations of MDS.
Asunto(s)
Antimetabolitos Antineoplásicos/uso terapéutico , Azacitidina/uso terapéutico , Enfermedades de la Piel/tratamiento farmacológico , Anciano , Anemia Refractaria con Exceso de Blastos/complicaciones , Cabeza , Humanos , Masculino , Cuello , Inducción de Remisión , Hombro , Enfermedades de la Piel/etiologíaRESUMEN
Sclerodermatous graft-versus-host disease (GVHD) is a rare complication of bone marrow transplantation. While GVHD is often associated with the beneficial graft vs. tumour effect, it also contributes towards significant morbidity and mortality. No reliably effective treatment has yet been established. We present 10 patients with haematological malignancies who underwent an allogeneic stem cell transplant and developed sclerodermatous GVHD. Donor lymphocyte infusion administered for relapse or reducing donor T-cell chimerism was a known trigger for sclerodermatous GVHD in four of the patients. Treatment with immunosuppressants, psoralen plus ultraviolet A (PUVA) and extracorporeal photopheresis has been largely unsuccessful in their management. Intensive immunosuppression including the use of anti-CD20 monoclonal antibody may have contributed to relapse of leukaemia in one patient 10 years after her transplant. Sclerodermatous GVHD may occur without a preceding lichenoid stage. Clinical heterogeneity is common, although sclerodermatous GVHD has a predilection for the limbs. Treatment options are largely unsatisfactory if conventional immunosuppression fails. PUVA may give some symptomatic benefit and extracorporeal photopheresis seems to be less efficacious than previously published work suggests.
Asunto(s)
Esclerodermia Localizada/terapia , Adulto , Anciano , Anemia Refractaria/cirugía , Trasplante de Médula Ósea/efectos adversos , Femenino , Enfermedad de Hodgkin/cirugía , Humanos , Inmunosupresores/uso terapéutico , Leucemia Mieloide/cirugía , Masculino , Persona de Mediana Edad , Terapia PUVA , Plasmacitoma/cirugía , Recurrencia , Esclerodermia Localizada/clasificación , Esclerodermia Localizada/etiología , Trasplante de Células Madre/efectos adversos , Trombocitosis/cirugía , Insuficiencia del TratamientoAsunto(s)
Carcinoma Basocelular/radioterapia , Neoplasias Inducidas por Radiación/radioterapia , Neoplasias Cutáneas/radioterapia , Anciano de 80 o más Años , Carcinoma Basocelular/etiología , Femenino , Humanos , Neoplasias Inducidas por Radiación/etiología , Radioterapia/efectos adversos , Neoplasias Cutáneas/etiología , Tiña del Cuero Cabelludo/radioterapia , Resultado del TratamientoRESUMEN
We describe a patient with erythrodermic adult T-cell leukaemia/lymphoma resistant to multiple systemic therapies who, on the commencement of daclizumab, a humanized anti-interleukin-2 receptor antibody, developed a rapid and sustained complete response with resolution of previously debilitating erythroderma, suggesting significant activity of this agent in this disease process.
Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Antineoplásicos/uso terapéutico , Inmunoglobulina G/uso terapéutico , Inmunosupresores/uso terapéutico , Síndrome de Sézary/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados , Daclizumab , Resultado Fatal , Humanos , Masculino , Persona de Mediana Edad , Síndrome de Sézary/patología , Neoplasias Cutáneas/patologíaRESUMEN
Graft-versus-host disease (GvHD) is a common sequel to allogeneic bone marrow transplants, which may be accompanied by desirable graft-versus-tumour effects. Sclerodermatous GvHD is a rare subtype that is very difficult to treat. We report the first case of sclerodermatous GvHD as part of the Koebner phenomenon. We propose that donor lymphocyte infusion and interferon-alpha were involved in the pathogenesis of this case.
Asunto(s)
Antineoplásicos/efectos adversos , Enfermedad Injerto contra Huésped/etiología , Interferón-alfa/efectos adversos , Transfusión de Linfocitos/efectos adversos , Esclerodermia Localizada/etiología , Adulto , Erupciones por Medicamentos/etiología , Erupciones por Medicamentos/patología , Enfermedad Injerto contra Huésped/inducido químicamente , Enfermedad Injerto contra Huésped/patología , Enfermedad de Hodgkin/terapia , Humanos , Masculino , Esclerodermia Localizada/inducido químicamente , Esclerodermia Localizada/patologíaRESUMEN
Granulocyte colony-stimulating factor (GCSF) is a recombinant human growth factor widely used in haematology. It is known to cause cutaneous vasculitis and neutrophilic dermatoses. We present three cases of Sweet's syndrome (SS) associated with GCSF use. Raised GCSF levels have been demonstrated in patients with SS. GCSF is the best understood mechanism by which neutrophil accumulation occurs and shows a dose-dependent effect in provoking SS.
Asunto(s)
Erupciones por Medicamentos/etiología , Factor Estimulante de Colonias de Granulocitos/efectos adversos , Síndrome de Sweet/inducido químicamente , Adyuvantes Inmunológicos/efectos adversos , Adulto , Femenino , Filgrastim , Humanos , Lenograstim , Persona de Mediana Edad , Proteínas Recombinantes/efectos adversosRESUMEN
All types of leukaemia can disseminate to the skin, producing cutaneous deposits known as leukaemia cutis (LC). We undertook a retrospective study to review the clinical presentations, treatment and outcome of eight patients with LC managed in our department over a period of 12 years. The clinical phenotype varied, with erythematous papules and nodules occurring with greatest frequency. Infiltrated haemorrhagic plaques and perifollicular acneiform papules were also seen. Although patients were treated aggressively for their underlying leukaemia, and received therapy directed towards LC, they tended to be refractory to treatment and the diagnosis was generally associated with a poor prognosis. The exception was a patient with chronic lymphocytic leukaemia, who survived 3 years after developing LC.
Asunto(s)
Leucemia Linfocítica Crónica de Células B/patología , Leucemia Mieloide/patología , Infiltración Leucémica/terapia , Piel/patología , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
The aim of this study was to examine trends in the presentation of cutaneous malignant melanoma at King's College Hospital (KCH) over the last three decades (1970-2000). KCH was one of seven centres that participated in the 1987 Cancer Research Campaign (CRC) publicity campaign aimed at promoting earlier self-recognition of melanoma. Data included patient age at presentation, sex, tumour site, Breslow thickness and histological subtype. The late 1980s saw a threefold increase in the annual number of melanomas and an eightfold increase in thin melanomas compared to the 1970s. The increase occurred in both sexes and was particularly marked after the CRC campaign but numbers had already begun to increase prior to this. The increase has predominantly been thin (Breslow < 1.5 mm) tumours of the superficial spreading variety with a resultant fall in mean Breslow thickness. There has been a decline in the annual number of melanomas since the peak in 1992 which is not explained by increased proportion of in situ tumours. The CRC campaign may have contributed to the documented increase in thin tumours but this trend had begun prior to 1987 suggesting factors other than public awareness and earlier presentation are important. It is encouraging that the number of melanomas has declined over the last 5 years at KCH but it is yet to be seen whether this reflects a real decrease in the incidence of melanoma.
Asunto(s)
Melanoma/epidemiología , Neoplasias Cutáneas/epidemiología , Neoplasias Abdominales/epidemiología , Distribución por Edad , Brazo , Femenino , Neoplasias de Cabeza y Cuello/epidemiología , Humanos , Pierna , Londres/epidemiología , Masculino , Melanoma/patología , Persona de Mediana Edad , Prevalencia , Distribución por Sexo , Neoplasias Cutáneas/patologíaRESUMEN
Paroxysmal nocturnal haemoglobinuria is a clonal stem cell disorder manifesting as haemolysis, bone marrow failure and thrombosis. We report a rare cutaneous manifestation on the trunk of a 40-year-old patient. Skin biopsy revealed the presence of fibrin thrombi in the capillaries. The eruption responded within days after commencing oral corticosteroids.
Asunto(s)
Hemoglobinuria Paroxística/complicaciones , Hemorragia/etiología , Enfermedades Cutáneas Vesiculoampollosas/etiología , Adulto , Biopsia/métodos , Hemoglobinuria Paroxística/patología , Hemorragia/patología , Humanos , Masculino , Enfermedades Cutáneas Vesiculoampollosas/patologíaAsunto(s)
Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Piperazinas/toxicidad , Pirimidinas/toxicidad , Enfermedades de la Piel/inducido químicamente , Anciano , Antineoplásicos/administración & dosificación , Antineoplásicos/uso terapéutico , Antineoplásicos/toxicidad , Benzamidas , Relación Dosis-Respuesta a Droga , Femenino , Histocitoquímica , Humanos , Mesilato de Imatinib , Leucemia Mielógena Crónica BCR-ABL Positiva/complicaciones , Masculino , Persona de Mediana Edad , Piperazinas/administración & dosificación , Piperazinas/uso terapéutico , Pirimidinas/administración & dosificación , Pirimidinas/uso terapéutico , Enfermedades de la Piel/patologíaRESUMEN
Congenital erythropoietic porphyria is a rare autosomal recessive disorder of haem biosynthesis caused by a deficiency of uroporphyrinogen III synthetase. There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. Accumulation of porphyrins results in haemolysis and extensive photosensitivity. The consequences of chronic haemolysis are splenomegaly, reactive erythroid hyperplasia, erythrodontia, bone fragility, extreme photosensitivity and photomutilation. We present a 35-year-old man who has the severe infantile form and illustrates the haematological and photodestructive complications despite attempts at treatment with hypertransfusion, oral charcoal therapy and beta-carotene. Allogenic bone marrow transplantation has been considered but because of the high associated mortality this procedure has been discounted at present in the management of our patient.
Asunto(s)
Porfiria Eritropoyética/terapia , Adulto , Anemia Hemolítica/etiología , Anemia Hemolítica/terapia , Transfusión Sanguínea , Enfermedades Óseas/etiología , Deferoxamina/administración & dosificación , Femenino , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Quelantes del Hierro/administración & dosificación , Sobrecarga de Hierro/tratamiento farmacológico , Sobrecarga de Hierro/etiología , Porfiria Eritropoyética/complicacionesRESUMEN
BACKGROUND: Gulf war veterans report more symptomatic ill-health than other military controls, and skin disease is one of the most frequent reasons for military personnel to seek medical care. AIM: To compare the nature and prevalence of skin disease in UK Gulf veterans with non-Gulf veterans, and to assess whether skin disease is associated with disability. DESIGN: Prospective case comparison study. METHODS: Disabled (n=111) and non-disabled (n=98) Gulf veterans and disabled non-Gulf veterans (n=133) were randomly selected from representative cohorts of those who served in the Gulf conflict 1990-1991, UN Bosnia Peacekeeping Force 1992-1997, or veterans in active service between 1990-91, but not deployed to the Gulf. Disability was defined as reduced physical functioning as measured by the Short Form 36 [score <72.2]. All subjects recruited were examined by a dermatologist, blind to the military and health status of the veteran. RESULTS: The prevalences of skin disease in disabled Gulf, non-disabled Gulf and disabled non-Gulf veterans were 47.7, 36.7, and 42.8% respectively. Seborrhoeic dermatitis was twice as common as expected in the Gulf veterans (both disabled and non-disabled). DISCUSSION: Skin disease does not appear to be contributing to ill health in Gulf war veterans, with the exception of an unexplained two-fold increase in seborrhoeic dermatitis.