ABSTRACT
Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Subject(s)
Adult , Humans , Male , Biopsy , Exophthalmos/pathology , Exophthalmos/etiology , Exophthalmos/drug therapy , Glucocorticoids/administration & dosage , Magnetic Resonance Imaging , Panniculitis, Nodular Nonsuppurative/pathology , Panniculitis, Nodular Nonsuppurative/drug therapy , Panniculitis, Nodular Nonsuppurative/complicationsABSTRACT
Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Subject(s)
Adult , Humans , Male , Biopsy , Exophthalmos/pathology , Exophthalmos/etiology , Exophthalmos/drug therapy , Glucocorticoids/administration & dosage , Magnetic Resonance Imaging , Panniculitis, Nodular Nonsuppurative/pathology , Panniculitis, Nodular Nonsuppurative/drug therapy , Panniculitis, Nodular Nonsuppurative/complicationsABSTRACT
Os autores relatam um caso de amiloidose cutânea, localizada em uma paciente de 58 anos, associada a telangiectasia hemorrágica hereditária. Esta forma de amiloidose é rara, e apresenta as características histopatológicas e prognósticas diferentes das variantes liquenóide e maculosa. O aspecto peculiar deste caso é associaçäo das doenças, fato do nosso conhecimento näo foi referido na literatura
Subject(s)
Humans , Female , Middle Aged , Amyloidosis/complications , Amyloid/metabolism , Panniculitis, Nodular Nonsuppurative/complications , Telangiectasia, Hereditary Hemorrhagic/complications , Diabetes Mellitus/etiologyABSTRACT
Os autores relatam um caso de doença de Weber-Christian (paniculite nodular recidivante näo-esclerosante), associada a gota, näo descrita anteriormente na literatura sem resposta ao uso de indometacina e razoável resposta ao uso de prednisona
Subject(s)
Aged , Humans , Male , Gout/complications , Panniculitis, Nodular Nonsuppurative/complications , Allopurinol/therapeutic use , Gout/drug therapy , Indomethacin/therapeutic use , Panniculitis, Nodular Nonsuppurative/drug therapy , Prednisone/therapeutic useSubject(s)
Adult , Female , Antibodies, Antinuclear/immunology , Lupus Erythematosus, Systemic/immunology , Panniculitis, Nodular Nonsuppurative/complications , Autoantibodies , Fluorescent Antibody Technique , Lupus Erythematosus, Systemic/complications , Panniculitis, Nodular Nonsuppurative/pathologyABSTRACT
SE presenta el caso de un niño intervenido quirúrgicamente de urgencia con el diagnóstico de apendicitis aguda. En el acto quirúrgico se encontró una masa tumoral polilobulada que ocupaba el mesentario del ciego y parte del íleon terminal con abundantes adenopatías y toma del tejido adiposo que obligó a una resección ileocólica. Los estudios hísticos demostraron una paniculitis mesentérica (variedad poco frecuente del síndrome de Weber-Christian