Hypergammaglobulinemic purpura of Waldenström.

We report a case of a 33-year-old-woman with a one-year history of bilateral lower extremity vasculitis and laboratory evidence of hypergammaglobulinemia with otherwise unremarkable routine laboratory and rheumatologic studies. Her clinical picture, together with histopathologic evidence of leukocytoclastic vasculitis, favor a diagnosis of hypergammaglobulinemic purpura of Waldenström.

Combination therapy of prednisolone and mizoribine improves cryoglobulinemic vasculitis with purpura and skin ulcers.

Conventional treatment is not standardized for hepatitis C virus-negative cryoglobulinemia, but corticosteroids, immunosuppressive agents, and plasma exchange typically improved the symptoms. Mizoribine is an immunosuppressive agent that was developed in Japan and has been found to inhibit the proliferation of lymphocytes, especially B cells. We have encountered an elder patient who had hepatitis C virus-negative, type II cryoglobulinemic vasculitis with leg purpura and skin ulcers. Her symptoms improved and cryoglobulin disappeared by the combination therapy of prednisolone and mizoribine. We speculate the action mechanism of this therapy is due to immunosuppressive effects including up-regulation of the efficacy of prednisolone by mizoribine.

Hypergammaglobulinaemic purpura presenting as reticulate purpura.

Hypergammaglobulinaemic purpura, first described by Waldenstrom, is a rare skin disease. The essential features are the presence of purpura with polyclonal hypergammaglobulinaemia. We describe a case of hypergammaglobulinaemic purpura occurring in a Chinese man with reticulate purpura and haemorrhagic blisters.

[Purpura pigmentosa progressiva in type III cryoglobulinemia and tartrazine intolerance. A follow-up over 20 years]. / Purpura pigmentosa progressiva bei Kryoglobulinämie Typ III und Tartrazinintoleranz. Ein Verlauf über 20 Jahre.

A 58 year old patient with hepatitis virus C (HCV) infection had a secondary polyclonal IgG-IgM cryoglobulinemia with a benign 20 year course. Clinically the patient suffered from progressive pigmented purpura (PPP). Histologic evaluation revealed a lymphocytic vasculitis. Food containing tartrazine triggered flares of the PPP, as demonstrated with controlled oral provocation testing. In most of the previously described cases of HCV and type III cryoglobulinemia, the typical cutaneous finding was palpable purpura with leukocytoclastic vasculitis.

Hypergammaglobulinemic purpura associated with Sjögren's syndrome and chronic C type hepatitis.

Although hypergammaglobulinemic purpura usually occurs secondarily on the lower legs in several disorders, purpura has also recently been reported to be associated with chronic C type hepatitis (HCV). To define the differences in the clinical, histological and laboratory findings and the prognosis of hypergammaglobulinemic purpura associated with those two disorders, we examined ten patients with hypergammaglobulinemic purpura, 6 patients with hypergammaglobulinemic purpura associated with Sjögren's syndrome (SjS) and 4 patients with hypergammaglobulinemic purpura with chronic C type hepatitis. Five of 6 patients with SjS were female. Attacks of purpura occurred in the pretibial area in all cases. Triggering factors included long walks and prolonged standing. The mean duration of attacks was 6.4 days. No systemic manifestations were associated. Anti-Ro/SS-A and anti-La/SS-B antibodies were expressed in one case. Spontaneous regression was noted in all cases; however, recurrence was noted in one. On the other hand, all the 4 patients with hypergammaglobulinemic purpura associated with HCV were men. Purpura was indurated in a few cases. Involved sites included the knee, forearm, abdomen and thigh in addition to the lower leg. The mean duration of attacks was 12.6 days. Recurrent purpura was noted in one case. Cryoglobulin was positive in three cases. In one patient with severe recurrent purpura, attacks stopped with prednisolone 10 mg/day. Histologically, leukocytoclastic vasculitis was detected in three cases associated with SjS and two cases with HCV. In conclusion, hypergammaglobulinemic purpura associated with HCV appears to occur unilaterally with a sex predilection for men and the manifestations last longer than those associated with SjS. Severe palpable purpura was also noted in association with HCV; systemic prednisolone resulted in good control.

Hypergammaglobulinaemic purpura of Waldenström and Ro/SSA autoantibodies.

Seven out of nine patients (78%), seen over a 13-year period with hypergammaglobulinaemic purpura of Waldenström were found to have antibodies to Ro/SSA. Over this period we saw 175 patients who had antibodies to Ro/SSA. In six of the seven patients, associated diseases were recognized. Five had Sjögren's syndrome, and one had systemic lupus erythematosus with Sjögren's syndrome and thyroiditis (and died 19 years after initial presentation from cerebral infarction). Screening for antibodies to Ro/SSA is important in the diagnosis of patients with hypergamma-globulinaemic purpura, and is helpful in predicting prognosis.

Macroglobulinosis cutis / Macroglobulinosis cutis

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

Macroglobulinosis cutis / Macroglobulinosis cutis

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

Hypergammaglobulinemic purpura of Waldenström.

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.

Purpuric rashes in cystic fibrosis.

Sixteen patients with cystic fibrosis experienced vasculitic rashes of the lower extremities that resembled hypergammaglobulinemic purpura. The rashes were associated with increased serum gammaglobulin G levels and rather severe lung disease. The rashes are probably the expression of chronic lung infection and high antigenic load in these patients. Their appearance was associated, in most cases, with poor long-term survival.

Benign hyperglobulinemic purpura of Waldenström: a report of seven patients and long-term follow-up

Desde el reporte original (1943), se han descrito 150 casos de la púrpura hiperglobulinnémica de Waldenström. Dicha condición se caracteriza por la recurrencia de lesiones purpúricas en las extremidades inferiores de mujeres jóvenes. Concomitantemente en estos pacientes se encuentra: prolongación sustancial del tiempo de sedimetnación de los hematies, altos niveles de factor reumatoideo, marcado aumento de las inmunoglobulinas , complejos de antigeno-anticuerpo circulantes y pruebas de coagulación sanguínea normales. Se ha descrito una alta coincidencia con las colagenosis. Aquí presentamos nuestra experiencia de 18 años con 7 pacientes con este diagnóstico. Todos los pacientes son mujeres, con comienzo de su mal a una edad joven. Sus pruebas de coagulación fueron normales, la velocidad de sedimentación de hematies fue uniformemente alta, las inmunoglobulinas (especialmente IgG) mostraron aumento significativo y la elevación del factor reumatoideo fue notable en todas. Tres pacientes presentaron enfermedad de Sjögren, y dos lupus sistémico. Si hizo reevaluación en 1988 a cinco pacientes disponibles, la cual reveló: biopsias de piel con vasculitis leucocitoclástica, complejos antigeno-anticuerpo circulantes y positividad para anticuerpos contra SS A en todos los casos. Los estudios de linfocitos T fueron anormales y sugieren un problema en el balance inmunológico de estos pacientes. Se discute la patofisiología y el tratamiento

Peripheral corneal ulcer associated with benign hypergammaglobulinemic purpura.

We describe a 56-year-old woman with benign hypergammaglobulinemic purpura in whom scleritis, acute stromal keratitis and peripheral corneal furrowing developed. Initial therapy with topical antibiotics and steroids failed to control the scleritis and keratitis. Subsequent treatment with plasmapheresis led to a decrease in circulating immune complex and serum immunoglobulin levels, with resolution of the ocular inflammation. After 8 years of follow-up the serum IgG and IgM levels were again elevated; however, the corneal disease remained quiescent. Plasmapheresis may have a role in the therapy of this and other immune-complex-mediated ocular diseases.

Púrpura por hipergammaglobulinemias / Hypergammaglobulinemic purpura

Se presentan 4 casos de púrpura por hipergamaglobulinemia de Waldenstrom (PHG), caracterizados por brotes de púrpura palpable, preferentemente en miembros inferiores (MI). Suelen ser precedidos por discreto dolor, prurito o sensación de quemadura. Al retrogradar, dejan máculas pigmentarias y atróficas que dibujan un retículo característico. Se acompaña de artralgias, edema de MI, fiebre, ERS acelerada, anemia e hipergamaglobulinemia a predominio de IgG mono o policlonal, que define el cuadro. Histológicamente se encuentra una vasculitis leucocitoclásica en la fase aguda y linfomonocitaria en etapas tardias. En nuestra casuística, se comprobó en 3 casos la asociación con LES (2) y Hepatitis Crónica Activa (1). Un paciente no evidenció ninguna enfermedad de base. La inmunofluorescencia directa demostró depósitos de IgA, IgM, IgG y C en el 50%. Estos hallazgos, junto con altos títulos de F.R. encontrados, avalarían la posibilidad del daño vascular por acción de inmunocomplejos circulantes. La PHG fue descripta como entidad autónoma sin etiología; con posterioridad aparece asociada a numerosos proceso, por lo que se cuestiona la existencia real de la forma esencial o primitiva y tambien el grado de aumento de gamaglobulina necesario para categorizar la enfermedad (AU)

Púrpura por hipergammaglobulinemias / Hypergammaglobulinemic purpura

Se presentan 4 casos de púrpura por hipergamaglobulinemia de Waldenstrom (PHG), caracterizados por brotes de púrpura palpable, preferentemente en miembros inferiores (MI). Suelen ser precedidos por discreto dolor, prurito o sensación de quemadura. Al retrogradar, dejan máculas pigmentarias y atróficas que dibujan un retículo característico. Se acompaña de artralgias, edema de MI, fiebre, ERS acelerada, anemia e hipergamaglobulinemia a predominio de IgG mono o policlonal, que define el cuadro. Histológicamente se encuentra una vasculitis leucocitoclásica en la fase aguda y linfomonocitaria en etapas tardias. En nuestra casuística, se comprobó en 3 casos la asociación con LES (2) y Hepatitis Crónica Activa (1). Un paciente no evidenció ninguna enfermedad de base. La inmunofluorescencia directa demostró depósitos de IgA, IgM, IgG y C en el 50%. Estos hallazgos, junto con altos títulos de F.R. encontrados, avalarían la posibilidad del daño vascular por acción de inmunocomplejos circulantes. La PHG fue descripta como entidad autónoma sin etiología; con posterioridad aparece asociada a numerosos proceso, por lo que se cuestiona la existencia real de la forma esencial o primitiva y tambien el grado de aumento de gamaglobulina necesario para categorizar la enfermedad

The kidney in hypergammaglobulinemic purpura.

A 25-year-old woman with long-standing hypergammaglobulinemic purpura developed distal renal tubular acidosis and a urine-concentrating defect. The acidification defect was characterized as suggestive of impaired distal proton secretion by infusion of neutral phosphate. The concentrating defect was a form of acquired nephrogenic diabetes insipidus. On renal biopsy, IgM mesangial nephropathy was found along with multiple large hyaline tubular casts. The renal findings in hypergammaglobulinemic purpura are reviewed.

Myeloma, paraproteinemias, and the skin.

This review focuses on those systemic diseases or syndromes associated with monoclonal plasma cell disorders that may present with important cutaneous manifestations. Amyloidosis, POEMS syndrome, cutaneous plasmacytoma, xanthomas, benign hypergammaglobulinemic purpura of Waldenström, and scleromyxedema are emphasized.