Danazol for Henoch-Schonlein Purpura

TO THE EDITOR:

Henoch-Schonlein purpura is a form of generalized hypersensitivity vasculitis involving the skin, joints, kidneys, and gastrointestinal tract. Corticosteroids have been the mainstay of therapy [1]. We report the successful use of danazol in a young man with classic recurring Henoch-Schonlein purpura refractory to low-dose prednisone therapy [2].

A 22-year old white man in good health developed an upper respiratory infection in November 1988. A few days later, palpable small spots appeared over both malleoli and quickly spread to lower extremities and genitals. There were associated myalgias, arthralgias, and swelling of wrist and ankle joints, followed by lower abdominal pain with nausea, protracted vomiting, and passage of tarry stools. Urinalysis showed numerous red blood cells, red cell casts, and mild proteinuria. Findings in a skin biopsy specimen were consistent with leukocytoclastic vasculitis, and the diagnosis of Henoch-Schonlein purpura was made.

The patient was begun on prednisone, 20 mg thrice daily, and ranitidine, 150 mg twice daily, with resolution of his gastrointestinal symptoms, hematuria, and proteinuria. However, each time prednisone therapy was discontinued, the symptoms recurred. At a prednisone dosage of 20 mg/d, he was free from hematuria but not from the red spots on lower extremities. Because of side effects of prednisone (acne, cushingoid features, weight gain, and emotional instability), danazol was begun at 400 to 600 mg/d in November 1989, and prednisone was tapered and then stopped at 4 weeks. Clearing of red spots was noticed after 2 weeks of danazol therapy. Except for one short relapse after an upper respiratory infection, he was in complete remission from Henoch-Schonlein purpura for 6 months while on danazol alone. However, when danazol was reduced to 200 mg on alternate days, the red spots reappeared on his legs, as did the hematuria, with both clearing when danazol dosage was increased. After 1 year of treatment, danazol was gradually reduced to 200 mg on alternate days. Occasional red spots on lower extremities and microscopic hematuria were observed in association with upper respiratory infection. He is now on danazol, 200 mg once or twice a week. His Henoch-Schonlein purpura has been in remission now for 3 years without major attacks. He has had no major side effects from danazol.

Henoch-Schonlein purpura is often a self-limiting disorder [1], but recurrences are quite common after the first attack. It is unlikely that spontaneous remission was responsible for the favorable outcome in our patient because his disease recurred each time prednisone therapy was discontinued and also later when danazol dosage was reduced.

Danazol has been used with success for IgA nephropathy (Berger disease), a disorder with clinicopathologic features similar to those seen with Henoch-Schonlein purpura [3]. The drug has diverse biologic properties, including immune modulation [4, 5], that may account for the apparent therapeutic effect, and it should be considered for the treatment of recurrent, chronic, or severe forms of Henoch-Schonlein purpura.

• Copyright 2004 by the American College of Physicians