RESUMEN
Background: Mast cells are immune cells that mediate hypersensi-tivity and allergic reactions in the body, secreting histamine and other inflammatory molecules. They have been associated with different inflammatory conditions such as obesity and other adipose tissue di-sorders. Lipedema is a chronic disease characterized by an abnormal accumulation of adipose tissue on the legs and arms, pain, and other symptoms. Mast cells may play a role in the pathology of lipedema. Objective: Pilot study to determine levels of histamine and its metabolites in lipedema subcutaneous adipose tissue (SAT) biopsy samples, and to test sodium cromoglycate for the treatment of mast cells in women with lipedema. Methods: Biopsies from lipedema and control SAT were collected and analyzed histologically for the presence of mast cells. Mass spec-trometry was used to measure the levels of histamine, a key marker of mast cells, and its metabolites in SAT in women with lipedema and controls, and after a group of women with lipedema were administered oral and topical doses of sodium cromoglycate for two weeks. Results: Histological examination of biopsies from lipedema patients confirmed the presence of mast cells. Metabolomic analysis revealed high levels of histamine and its metabolites in samples from women with lipedema compared to controls. Following a two-week treatment period, lipedema tissue samples exhibited reduced levels of histamine, suggesting a reduction of mast cell activity. Conclusion: Sodium cromoglycate has the ability to stabilize mast cells and reduce histamine levels in lipedema patients, which could be useful in lowering the symptoms of lipedema.
Asunto(s)
Lipedema , Humanos , Femenino , Lipedema/tratamiento farmacológico , Lipedema/metabolismo , Lipedema/patología , Cromolin Sódico/uso terapéutico , Cromolin Sódico/metabolismo , Mastocitos/metabolismo , Mastocitos/patología , Histamina/metabolismo , Proyectos PilotoRESUMEN
Chronic oedema is a term that encompasses several causes that lead to oedema formation in any part of the body. This includes lymphoedema and its subcategories. Despite the assumption that these are different, there are more commonalities amongst them. This makes the transfer of knowledge and experience, from chronic oedema conditions to lymphoedema, and vice versa easier to achieve. However, this takes both time and support, to achieve the ultimate patient outcome of self-care, with generalist able to support this process and journey.
Asunto(s)
Edema/clasificación , Edema/diagnóstico , Edema/tratamiento farmacológico , Lipedema/clasificación , Lipedema/diagnóstico , Lipedema/tratamiento farmacológico , Crema para la Piel/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Enfermedad Crónica/clasificación , Enfermedad Crónica/terapia , Femenino , Humanos , Masculino , Persona de Mediana Edad , Evaluación de Síntomas/métodosAsunto(s)
Lipedema/patología , Dermatosis del Cuero Cabelludo/patología , Antiinflamatorios/uso terapéutico , Diagnóstico Diferencial , Medicina Basada en la Evidencia , Femenino , Humanos , Lipedema/tratamiento farmacológico , Persona de Mediana Edad , Dermatosis del Cuero Cabelludo/tratamiento farmacológico , Resultado del TratamientoRESUMEN
Neutrophilic dermatosis (ND) confined to postmastectomy lymphedema, localized Sweet syndrome, is a newly recognized disease. In this study, the authors describe a 44-year-old obese woman with chronic myelogenous leukemia in molecular remission on dasatinib therapy, who presented with a painful urticarial eruption limited to lipo-lymphedematous skin and accompanied by malaise, episodic fever, diarrhea, neutrophilia, and leukocytosis. Initially transient and migratory, the rash became fixed, papular, and vesicular and showed minimal response to corticosteroids. Biopsy demonstrated sparse perivascular and interstitial dermal neutrophilic infiltrates, without vasculitis or significant dermal edema. Aggregates of neutrophils were found within and surrounding lymphangiectases. Biopsy of a new onset papule 3 weeks later demonstrated papillary dermal edema, denser neutrophilic infiltrate, and vasculitis-like changes. These 2 histopathologic patterns of ND, early and late, resemble neutrophilic urticarial dermatitis (also known as neutrophilic dermatitis with systemic inflammation) and Sweet syndrome, respectively. Extensive workup did not reveal evidence of relapsed chronic myelogenous leukemia, infection, or a coexisting systemic inflammatory disease. Dasatinib was discontinued and the eruption gradually resolved over 2.5 months. Still in molecular remission (no detectable BCR-ABL gene fusion), dasatinib therapy was recommenced at 3-month follow-up. After 10 months, she complains of malaise and arthralgia, but no cutaneous symptoms. The evolution and slow resolution of this ND in lipo-lymphedematous skin implicate poor lymphatic clearance of factors, antigenic and/or toxic, involved in the pathogenesis of ND.
Asunto(s)
Antineoplásicos/efectos adversos , Dasatinib/efectos adversos , Erupciones por Medicamentos/etiología , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Lipedema/inducido químicamente , Linfedema/inducido químicamente , Infiltración Neutrófila/efectos de los fármacos , Obesidad Mórbida/complicaciones , Piel/efectos de los fármacos , Síndrome de Sweet/inducido químicamente , Adulto , Antiinflamatorios/uso terapéutico , Biopsia , Progresión de la Enfermedad , Erupciones por Medicamentos/diagnóstico , Erupciones por Medicamentos/tratamiento farmacológico , Femenino , Humanos , Leucemia Mielógena Crónica BCR-ABL Positiva/complicaciones , Leucemia Mielógena Crónica BCR-ABL Positiva/diagnóstico , Lipedema/diagnóstico , Lipedema/tratamiento farmacológico , Linfedema/diagnóstico , Linfedema/tratamiento farmacológico , Obesidad Mórbida/diagnóstico , Piel/patología , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/tratamiento farmacológico , Resultado del TratamientoRESUMEN
Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.