HYPERGAMMAGLOBULINEMIC PURPURA OF WALDENSTRÖM

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.

Clinical

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.

Treatment

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.

References

Hypergammaglobulinemic purpura of Waldenström. Dermatology Online Journal 18(12)

RegionalDerm

Home | FAQs | Use of Images | Privacy Policy | Contact


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.