Persistent and progressive purpura in a patient with an elevated rheumatoid factor and polyclonal gammopathy (hypergammaglobulinemic purpura of Waldenström)

Hypergammaglobulinemic purpura: Does hypergammaglobulinemia cause purpura?

“…Dear Editor, Hypergammaglobulinemic purpura (HP) of Waldenström is a syndrome characterized by recurrent lower extremity purpura with hypergammaglobulinemia. 1 HP can be a primary disease, but is sometimes associated with other diseases, such as Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and hepatitis C. 1 It has been hypothesized that high levels of circulating immunoglobulins (Ig) are associated with HP pathogenesis via immune complex formation and deposition in the vessel walls. 2 However, evidence showing that hypergammaglobulinemia directly causes HP is absent.…”

Livedoid Vasculitis in a Patient With Sjogren Syndrome Successfully Treated With Methotrexate

“…5 The condition can be effectively managed with compression stockings, avoidance of prolonged standing, and hydroxychloroquine. 6 , 7 Patients who are refractory to these regimens or who have more severe symptoms may require oral prednisolone. Individuals with treatment-refractory attacks are treated with azathioprine or intravenous (IV) Rituximab at doses typically used to treat RA.…”

Hypergammaglobulinemic purpura of Waldenström—Unusual and impressive case in a patient with myeloma: A case report

“…HGPW may be primary or secondary, and in older adults, it is often secondary to an underlying immune dysregulation or granulomatous condition. 1 Sjögren syndrome is the commonest association; however, rare occurrences of lymphoma or myeloma have been reported. 1,3–8 A common link between these disease entities is polyclonal immunoglobin formation with immune complex deposits in blood vessels of the lower limbs.…”

“…1,3–8 A common link between these disease entities is polyclonal immunoglobin formation with immune complex deposits in blood vessels of the lower limbs. 1 The differential of HGPW includes non-inflammatory purpuras like pigmented purpuric dermatosis, as well as other forms of LCV. Herein is presented an unusually florid case of HGPW, associated with myeloma, that nevertheless resolved spontaneously in several days.…”

“…Hypergammaglobulinemic purpura of Waldenström (HGPW) is characterized by recurrent purpura on the lower extremities, increased erythrocyte sedimentation rate (ESR), and polyclonal gammopathy. 1 The syndrome is primarily seen in middle-aged women and may be brought on by prolonged increases in hydrostatic pressure including heat exposure, tight-fitting clothing, and extended periods of standing. 2 It is typically mild and self-limiting and is considered an unusual form of chronic leukocytoclastic vasculitis (LCV).…”