By Gary M. White, MD
Hypergammaglobulinemic purpura of Waldenstrom is a benign purpuric rash that usually affects the lower legs of middle-aged women. To this author's view, it seems very similar to exercise-induce purpura and in fact may just be variants of the same process.
The classic patient develops a shower of purpuric lesions, palpable or non-palpable, on the legs after a significant period of standing or walking. Occasionally, the rash may affect the trunk. Sjögren's syndrome or other vasculitis is sometimes associated. A biopsy may show hemorrhage, a mild perivascular lymphocytic infiltrate, or leukocytoclastic vasculitis.
In the primary form which typically affects young women, a positive SSA may be found and there is a risk for fetal complications during pregnancy including fetal demise. Thus, in women, an inquiry should be made about symptoms of Sjögren's syndrome and SSA/SSB should be checked.
The condition is benign, so aggressive treatment is usually not warranted. Triggers should be avoided. Support hose may be helpful. Aspirin, indomethacin, dipyridimole, and hydroxychloroquine have been used. There are case reports of success with colchicine, mycophenolate mofetil, and rituximab.
Hypergammaglobulinemic purpura of Waldenström. Dermatology Online Journal 18(12)