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THU0218 Successful treatment of weber christian disease (wcd) with thalidomide in a patient failing multiple other medical therapies
  1. HR Barthel
  1. Koenigstein, Germany

Abstract

Objectives A case of idiopathic nodular panniculitis (WCD) with recurrent febrile episodes resistant to corticosteroids and methotrexate (MTX) in alternating combin. with hydroxychloroquine (HCQ), azathioprine, cyclosporine, colchicine, and doxycycline is described. Thalidomide 50–100 mg/d induced a remission – now since 2y.

Methods CASE REPORT:The patient is a 65y.o. German male who presented first in 3/1998 with a 2-y. hist. of recur. febr. attacks with 1–2 cm, painful, palp., subc., non-draining, eryth. nodules occurr. on lower legs, backside of upper thighs, lower back, and upper arms. 3 consec. biop. showed lobular and septal panniculitis. Lab. exam. revealed mild leukopenia (2500/mm3), mild thrombocytopenia, CRP 2.5 mg/dl, sed.-rate 50 mm/h, ferritin 900 ng/ml. Blood cultures, ANA, ANCA, RF, C3, C4, C1q-Inhib., cardiolip.-AB, immunoelpho., borr. burgd. ser., a1-antitryp., serum amyl. and lip., and UA were all normal or neg. Bone marrow biopsy showed unspecific changes. Chest x-ray was normal. Abdominal CT gave no hint for lymphoma or malignancy. Despite 25 mg/d of prednisolone and 20 mg weekly MTX recurrent attacks occurred. Various drugs were tried in combination with MTX in order to lower steroids such as azathioprine 100 mg/d, HCQ 400 mg/d, doxycyc. 200 mg/d, colchicine 1.2 mg/d, and cyclosporine (200 mg/d), all without improvement.

In 3/1999 sympt. worsened and CRP rose intermitt. to 16 mg/dl, further lab. exam. showed persistence of leukopenia 3400/mm3, normal platelet counts, but drop of haemoglobin to 9.5 without clinical evidence of a GI-bleed. A new bone marrow biopsy revealed lymphocytic hyperplasia with no histol. or cytol. support for erythrophagocytosis.

Thalidomide initially 150 mg/d at night, then after 2 weeks at 100 mg/d was started and induced disappearance of febrile attacks and subcutaneous nodules. CPR, sedimentation-rate, and haematological findings normalised. Glucocorticosteroids and MTX were discontinued. After 3 months thalidomide was reduced to 50 mg/d with infrequent mild relapses (with mild subcutaneous nodules only), which disappear within 1–2 day with temporary thalidomide dose increase to 100 mg/d.

Conclusion Rarity of WCD has so far hampered a controlled therapeutic trial with any drug. Only case reports with various drugs exist suggesting mostly corticosteroids, MTX, HCQ, cyclosporine, which all failed in this case. The use of thalidomide in WCD is suggested by clinical similarities to erythema nodosum leprosum and lupus profundus panniculitis, immunologic mediated conditions responding well to thalidomide. There has been so far only one other report of thalidomide use in WCD. 24 years ago a favourable response in a 23 y.o. Female from Malaysia was reported. This patient had a remission, which was ongoing despite stopping of all medications after treatment with thalidomide of only 13 weeks. The question of a possible spontaneous remission asked by the describing authors remains unanswered. To the contrary, in the here described case repeated reduction of thalidomide below 100 mg/dl caused mild recurrence of sympt., responding immediately to dose increase to 100 mg.

Thalidomide?s anti-TNFa modulatory and anti-angiogenic effects have recently been rediscovered also for treatment of other autoimmune diseases. Superior tolerability and side-effect profile (compared to corticosteroids and immunosuppressives) make thalidomide a first line treatment consideration for chronic WCD in patients without childbearing potential.

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