Subject(s)
Myositis/complications , Purpura, Hyperglobulinemic/complications , Anti-Inflammatory Agents/therapeutic use , Diagnosis, Differential , Female , Humans , Leg/pathology , Middle Aged , Myositis/diagnosis , Myositis/drug therapy , Myositis/pathology , Purpura, Hyperglobulinemic/diagnosis , Purpura, Hyperglobulinemic/drug therapy , Purpura, Hyperglobulinemic/pathology , Steroids/therapeutic useSubject(s)
Plasma Cells/pathology , Purpura, Hyperglobulinemic/diagnosis , Skin Diseases/diagnosis , Waldenstrom Macroglobulinemia/diagnosis , Adult , Humans , Macroglobulins/metabolism , Male , Purpura, Hyperglobulinemic/metabolism , Skin Diseases/metabolism , Waldenstrom Macroglobulinemia/metabolismABSTRACT
Hypergammaglobulinemic purpura of Waldenström is a rare syndrome that includes recurrent episodic purpura occurring mainly on the lower extremities and dorsum of the feet. The hallmark of this condition is polyclonal hypergammaglobulinemia primarily composed of IgG. Although the condition generally is benign, it may herald an underlying connective tissue disease or hematologic malignancy. We report a case of a 47-year-old woman with episodic purpura of 3 years' duration associated with Raynaud phenomenon.
Subject(s)
Immunoglobulin G/blood , Purpura, Hyperglobulinemic/immunology , Raynaud Disease/complications , Female , Follow-Up Studies , Humans , Middle Aged , Purpura, Hyperglobulinemic/diagnosis , Purpura, Hyperglobulinemic/etiologyABSTRACT
A 43-year-old healthy patient developed disseminated flat skin-colored to red-brown papules over a few months. These papules were the result of cutaneous IgM deposits representing the first symptom of a hitherto undiagnosed IgM paraproteinemia. This extremely rare skin manifestation of IgM paraproteinemia e. g. possibly incipient Waldenström macroglobulinemia should be included in the histopathological differential of eosinophilic dermal deposits.
Subject(s)
Immunoglobulin M/immunology , Paraproteinemias/diagnosis , Paraproteinemias/therapy , Purpura, Hyperglobulinemic/diagnosis , Purpura, Hyperglobulinemic/therapy , Adult , Diagnosis, Differential , Humans , Macroglobulins , Male , Paraproteinemias/immunology , Purpura, Hyperglobulinemic/immunology , Treatment OutcomeABSTRACT
A woman presented at 25 weeks gestation in her first pregnancy with severe preeclampsia and an intrauterine death. It later emerged that she had Waldenstrom's benign hypergammaglobulinemic purpura. We discuss the implications of this diagnosis in pregnancy and explore possible management options during subsequent pregnancies.
Subject(s)
Pregnancy Complications, Cardiovascular/diagnosis , Pregnancy Complications, Hematologic/diagnosis , Purpura, Hyperglobulinemic/diagnosis , Adult , Female , Fetal Death , Humans , Pre-Eclampsia/etiology , Pregnancy , Purpura, Hyperglobulinemic/complicationsABSTRACT
Visceral leishmaniasis may present with cytopenias along with the formation of many autoantibodies and, rarely, with the presence of mixed cryoglobulinemia, type II, resembling an autoimmune disease. The syndrome of mixed cryoglobulinemia is characterized by the triad of purpura, arthralgias, and asthenia, in conjunction with cryoglobulins in the serum. In this article mixed cryoglobulinemia, type II, was diagnosed in a negative for hepatitis B or C patient suffering from visceral leishmaniasis. Antimicrobial therapy against leishmania eliminated the cryoglobulin titer, as well as the clinical manifestations of cryoglobulinemia. The role of the immune system and the type of immune response for the formation of cryoglobulins are discussed.
Subject(s)
Cryoglobulinemia/diagnosis , Leishmaniasis, Visceral/diagnosis , Purpura, Hyperglobulinemic/diagnosis , Aged, 80 and over , Amphotericin B/therapeutic use , Antiprotozoal Agents/therapeutic use , Bone Marrow/parasitology , Bone Marrow/pathology , Cryoglobulinemia/parasitology , Humans , Leishmaniasis, Visceral/complications , Liposomes , Male , Purpura, Hyperglobulinemic/parasitology , Treatment OutcomeABSTRACT
La neurocisticercosis es una afectación del sistema nervioso central por las larvas de la Taenia solium. Aunque en nuestro país su diagnóstico era excepcional, en los últimos años se ha observado un notable incremento en el número de casos diagnosticados, debido al fenómeno de la inmigración desde países donde la enfermedad es endémica. La forma de presentación más frecuente de la neurocisticercosis es la crisis epiléptica, seguida de la cefalea. Para el diagnóstico de sospecha debemos valorar los datos epidemiológicos y la clínica y confirmarlo mediante los estudios de neuroimagen e inmunológicos. El tratamiento de elección debe ser farmacológico, principalmente con albendazol, y reservar la cirugía para los casos en el que el primero falla. Las medidas higiénico-sanitarias y el tratamiento de los pacientes con teniasis son de suma importancia. La neurocisticercosis ha dejado de ser uno de esos diagnósticos excepcionales y dado el previsible aumento de su incidencia en nuestro medio, los profesionales sanitarios debemos conocer dicha enfermedad e incluirla en niveles más altos de los algoritmos de diagnóstico diferencial (AU)
Subject(s)
Adolescent , Adult , Female , Male , Middle Aged , Child , Humans , Cysticercosis/complications , Cysticercosis/diagnosis , Cysticercosis/therapy , Taenia/isolation & purification , Central Nervous System/pathology , Central Nervous System , Albendazole/administration & dosage , Albendazole/therapeutic use , Epilepsy/complications , Epilepsy/diagnosis , Neurologic Manifestations , Eosinophilia/diagnosis , Purpura, Hyperglobulinemic/complications , Purpura, Hyperglobulinemic/diagnosis , Diagnosis, Differential , Cysticercosis/drug therapy , Cysticercosis/prevention & control , Cysticercosis/epidemiologyABSTRACT
Se presenta un paciente de 60 años, argentino, con antecedentes de púrpura hipergammaglobulinémica, afectando miembros inferiores y abdomen, desde hace 19 años, con vasculitis leucocitoclástica, factor reumatoideo positivo y crioglobulinemia, asociada con cirrosis por hepatitis crónica por virus C. Es posible que pacientes diagnosticados como púrpura crioglobulinémica estén afectados de hipergammaglobulinas séricas y ambas púrpuras representen una misma enfermedad (AU)
Subject(s)
Humans , Male , Middle Aged , Purpura, Hyperglobulinemic/diagnosis , Hepatitis C, Chronic/complications , Purpura, Hyperglobulinemic/complications , Purpura, Hyperglobulinemic/classification , /complications , Lupus Erythematosus, Systemic/complications , Arthritis, Rheumatoid/complications , Liver Cirrhosis/complications , Multiple Myeloma/complications , Mikulicz' Disease/complicationsABSTRACT
Se presenta un paciente de 60 años, argentino, con antecedentes de púrpura hipergammaglobulinémica, afectando miembros inferiores y abdomen, desde hace 19 años, con vasculitis leucocitoclástica, factor reumatoideo positivo y crioglobulinemia, asociada con cirrosis por hepatitis crónica por virus C. Es posible que pacientes diagnosticados como púrpura crioglobulinémica estén afectados de hipergammaglobulinas séricas y ambas púrpuras representen una misma enfermedad
Subject(s)
Humans , Male , Middle Aged , Hepatitis C, Chronic/complications , Purpura, Hyperglobulinemic/diagnosis , Arthritis, Rheumatoid , Liver Cirrhosis/complications , Lupus Erythematosus, Systemic/complications , Multiple Myeloma/complications , Mikulicz' Disease , Purpura, Hyperglobulinemic/classification , Purpura, Hyperglobulinemic/complications , Sjogren's Syndrome/complicationsABSTRACT
Benign hypergammaglobulinemic purpura of Waldenström (HGPW) is an uncommon cause of non-thrombocytopaenic purpura that may create diagnostic difficulties. The presence of constitutional symptoms associated with prominent immunological abnormalities may raise alarm, leading to extensive and often unnecessary investigations. This report describes 3 young women with HGPW. Clinical features were characterised by recurrent episodes of bilateral asymmetrical palpable purpuric lesions on the lower extremities that were precipitated by a prolonged increase in hydrostatic pressure (e.g. prolonged standing, tight stockings etc.) associated with constitutional features. In one patient the condition was secondary to Sjögren's syndrome with type IV renal tubular acidosis. Laboratory abnormalities included a persistently elevated erythrocyte sedimentation rate, marked polyclonal hypergammaglobulinemia, and high titers of rheumatoid factor and anti-nuclear antibody of the anti-SSA (anti-Ro)/anti-SSB(anti-La) subsets. This topic is reviewed briefly with the emphasis that in its 'primary' form this condition could be considered a 'benign' systemic immunoinflammatory disease that requires neither extensive investigations nor any aggressive form of therapy. Greater awareness of HGPW may increase the frequency of its diagnosis, especially in the patient group with non-thrombocytopenic purpura or the so-called cutaneous vasculitic syndromes with 'palpable purpura'.
Subject(s)
Purpura, Hyperglobulinemic/diagnosis , Waldenstrom Macroglobulinemia/diagnosis , Adolescent , Adult , Female , Humans , Purpura, Hyperglobulinemic/etiology , Sjogren's Syndrome/complications , Waldenstrom Macroglobulinemia/complications , gamma-Globulins/metabolismABSTRACT
We describe a child who initially presented with recurrent infections. He subsequently developed hypergammaglobulinemic purpura but responded well to corticosteroids and hydroxychloroquine.
Subject(s)
Infections/etiology , Purpura, Hyperglobulinemic/complications , Purpura, Hyperglobulinemic/diagnosis , Child, Preschool , Disease Progression , Humans , Hydroxychloroquine/administration & dosage , Immunoglobulin G/blood , Infections/diagnosis , Infections/drug therapy , Male , Prednisone/administration & dosage , Purpura, Hyperglobulinemic/drug therapy , Recurrence , Treatment OutcomeABSTRACT
A 31-year-old female is described who developed benign hypergammaglobulinaemic purpura and lymphoid interstitial pneumonitis concomitantly. High titre anti-nuclear antibodies were also noted. Several years previously, the patient had developed myasthenia gravis and multiple sclerosis. The present case is an example of multiple medical disorders characterized by immune dysregulation and represents the first reported associated of hypergammaglobulinaemic purpura with lymphoid interstitial pneumonitis.
Subject(s)
Lung Diseases, Interstitial/complications , Purpura, Hyperglobulinemic/complications , Purpura, Hyperglobulinemic/diagnosis , Adult , Biopsy , Female , Humans , Immunoglobulin G/biosynthesis , Immunoglobulins/blood , Lung/pathology , Multiple Sclerosis/complications , Myasthenia Gravis/complications , Skin/pathologyABSTRACT
Púrpura puede ser la manifestación inicial y algunas veces la única de una gammapatía policlonal. Se presenta el caso de una paciente con lesiones purpúricas en miembros inferiores con diagnóstico inicial de púrpura hiperglobulinémica de Waldenström, en quien se encontró mieloma múltiple como enfermedad subyacente
Subject(s)
Middle Aged , Humans , Female , Antibodies, Antinuclear , Multiple Myeloma/complications , Purpura, Hyperglobulinemic/diagnosis , Skin Diseases/etiologyABSTRACT
Presentamos un paciente de 74 años con una forma específica cutánea de macroglobulinemia de Waldenstrom, denominada macroglobulinosis cutis, la cual es de observación excepcional. Las lesiones cutáneas resultaron de gran ayuda para diagnosticar la enfermedad de base. Fue tratado con ciclos de clorambucil, prednisona y plasmaféresis, evolucionando de manera satisfactoria
Subject(s)
Humans , Male , Aged , /diagnosis , Skin Manifestations , Purpura, Hyperglobulinemic/diagnosis , Purpura, Hyperglobulinemic/pathology , /complications , /pathologyABSTRACT
Presentamos un paciente de 74 años con una forma específica cutánea de macroglobulinemia de Waldenstrom, denominada macroglobulinosis cutis, la cual es de observación excepcional. Las lesiones cutáneas resultaron de gran ayuda para diagnosticar la enfermedad de base. Fue tratado con ciclos de clorambucil, prednisona y plasmaféresis, evolucionando de manera satisfactoria
Subject(s)
Humans , Male , Aged , Waldenstrom Macroglobulinemia/diagnosis , Skin Manifestations , Waldenstrom Macroglobulinemia/complications , Waldenstrom Macroglobulinemia/pathology , Purpura, Hyperglobulinemic/diagnosis , Purpura, Hyperglobulinemic/pathologyABSTRACT
Hypergammaglobulinemic purpura of Waldenström is characterized by hypergammaglobulinemia, recurring purpura, an elevated erythrocyte sedimentation rate, and the presence of rheumatoid factor indicative of circulating immune complexes. There is a significant association with autoimmune diseases, especially Sjögren's syndrome and lupus erythematosus. Hypergammaglobulinemic purpura is considered primary when there is no other associated disease or secondary when associated with other diseases, usually autoimmune. Immune derangements are fundamental in its pathogenesis, although its cause is still unknown. Therapy is unrewarding and is probably unnecessary for this usually benign condition. Three cases are presented that are representative of patients with hypergammaglobulinemic purpura.
