Hypergammaglobulinemic purpura of Waldenström.

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.

Hypergammaglobulinemic purpura in two sisters with Sjogren's syndrome responding to colchicine treatment.

Purpura is the hallmark of hypergammaglobulinemic purpura (HP). It appears mainly after strenuous activity in the dependent areas of the body. Treatment is mostly symptomatic. Here we report two sisters with Sjogren's syndrome (SS) and HP who had a remarkable response to colchicine treatment.

Hypergammaglobulinemic purpura of Waldenström: report of 3 cases with a short review.

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'.

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.

Vasculite leucocitoclásica em paciente com artrite reumatóide clássica / Leucocitoclastic vasculitis in patient with classical rheumatoid arytheritis

Os autores apresentam um caso de artrite reumatóide (AR) clássica de ñ 15 anos de duraçäo que apresentou na evoluçäo vasculite leucocitoclásica. Fazem uma revisäo de literatura e discutem os mecanismos etiopatogênicos envolvidos

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.

Doença adjuvante humana após injeçäo de parafina e silicone: primeiro relato no Brasil / Human adjuvant disease after injection of paraffin and silicone: first report in Brazil

A estimulaçäo antigênica provocada pela injeçäo de substâncias estranhas, como a parafina líquida ou o silicone, pode desencadear um espectro de manifestaçöes auto-imunes. As colagenoses, mais freqüentemente a esclerodermia, estäo entre as mais descritas. Quando estäo presentes alguns elementos clínico-laboratoriais, mas näo há dados suficientes para definir uma colagenose, surge a entidade denominada doença adjuvante humana. É descrito um caso que apresentou poliartrite, fenômeno de Raynaud e púrpura higerglobulinêmica como complicaçäo de injeçäo prévia de parafina e silicone em procedimento estético para as mamas

Paralisia periódica hipocaliêmica, distúrbio da funçäo renal e púrpura hiperglobulinêmia no curso de síndrome de Sjögren: a propósito de um caso / Paralysis, hypokalemic periodic, renal function disorder and purpura hyperglobulinemical in the Sjogren's syndrome: a propose of a case

Os autores apresentam o caso de uma paciente de 24 anos sofredora de síndrome de Sjöen com púrpura hiperglobulinêmica, acidose tubular renal, diabetes insípido nefrogênico e paralisia periódica hipocaliêmica, que motivara suas internaçöes. Apresentava ainda miocardiopatia chagásica e neurocisticercose. Discutem o diagnóstico e revisam os aspectos principais da síndrome de Sjöen

Cutaneous manifestations of cystic fibrosis: possible role of cryoglobulins.

A 13-year-old girl with cystic fibrosis (CF), severe lung disease, purpura on the lower extremities and with elevated serum mixed cryoglobulins is described. The possible relationship of the purpura in CF with cryoglobulinaemia is discussed.