Panniculitis and liver disease (hepatic Weber Christian disease)

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To the Editor:

A 47 years non diabetic, normotensive female with sober habits was referred to hepatologist in May 2003 for fever and elevated liver enzymes. She was suffering from Psoriasis since last 15 years and taken treatments for first 5 years. In April 2003, she was diagnosed to have panniculitis on skin biopsy by dermatologist. In first week of May, she was investigated for pyrexia which included complete blood count, ESR, liver and renal function tests, CT scan of chest and abdomen, markers for autoimmunity, viral hepatitis markers, serum calcium and serum angiotensin converting enzyme assay. The only abnormalities found were ESR 30mm at the end of one hour, SGOT 275mU/ml, SGPT 373mU/ml and LDH 457mU/ml. Serum proteins were 7.9g% with albumin of 2.2g%. CT scan of abdomen showed diffuse fatty liver. Liver biopsy performed at this point of time showed diffuse fatty change with mild inflammation without granuloma or fibrosis. Patient developed right sided pleural effusion followed by ascites. Examination of the pleural fluid revealed total protein of 3.6g%. Red blood cells were 750/mm³, leukocytes 130/mm³ of which 85% were lymphocytes. Pleural fluid LDH was 385mU/ml. Pleural fluid culture did not grow any organisms and was negative for acid-fast bacilli. Ascitic fluid examination showed total protein 2.8g%, red blood cells 180/mm³, leukocytes 80/mm³ which were predominantly lymphocytes. Ascitic fluid amylase was 49mU/ml and culture for acid fast bacilli was negative. The patient was subjected to laparoscopy in which peritoneum showed multiple areas of fat necrosis and liver was grossly fatty which were confirmed histologically. The diagnosis of hepatic Weber Christian Disease was made and patient was suggested to take corticosteroid therapy. Patient did not take treatment for next 15 days and she worsened clinically and biochemically. Then she agreed to take the treatment and received combination of oral prednisolone 30mg/day and Mycofenolate (500mg twice a day). Within 15 days of treatment fever, jaundice and ascites disappeared with improvement in liver function test. A follow up after one and half months showed complete normal liver functions with disappearance of symptoms.

Several forms of panniculitis with systemic disease have been described which may involve liver, spleen, large and small intestine, peritoneum and heart [1]. However, only rare cases of significant hepatitis in relation to panniculitis have been described in literature [1].

Oram and Cochrane [2] first described liver involvement in Weber Christian disease in the form of steatohepatitis. Recently, Wasserman et al. [1] described a case of hepatic Weber Christian disease presenting with recurrent fever, subcutaneous abscesses and cellulitis that developed significant liver disease. Kumagai-Kurata et al. [3] reported a case of idiopathic lobular panniculitis presenting with fever and pleural effusion. Awareness and good clinicopathologic correlation is required for the diagnosis of liver involvement in Weber Christian disease since it represents severe systemic form of panniculitis which requires intensive immunosuppressive therapy. Most of cases respond to corticosteroid therapy. In some cases, intensive immunosuppressive therapy with steroid sparing drugs is required [1]. Azathioprine, mycophenolate, cyclophospamide and cyclosporine have been used in few patients with beneficial results [1]. Enk et al. [4] used mycophenolate mofetil in 3 patients of relapsing idiopathic nodular Panniculitis. In all the 3 patients remission was induced by steroids but could not be maintained with Azathioprine or methotrixate, mycophenolate (2g/day) along with steroid showed remarkable improvement in 2 weeks. At later date, steroids were withdrawn and patients were maintained on 1g of mycophenolate. In 6–10 months follow up there was no relapse. Due to these results, we decided to use mycophenolate along with steroids and were able to induce remission and reduce steroid doses successfully in our patient.

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References 

1. Wasserman JM, Thung SN, Berman R, Bodenheimer HC, Sigal SH. Hepatic Weber–Christian disease. Semin Liver Dis. 2001;21:115–118
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2. Oram S, Cochrane GM. Weber–Christian disease with visceral enlargement. Br Med J. 1958;281–284
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3. Kumagai-Kurata N, Kunitoh H, Nagamine-Nishizawa M, Watanbe K, Nakamura N. Idiopathic lobular panniculitis with specific pleural involvement. Eur Respir J. 1995;8:1613–1615
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4. Enk AH, Knop J. Treatment of relapsing idiopathic nodular Panniculitis (Pfeifer–Weber–Christian disease) with mycophenolate mofetil. J Am Acad Dermatol. 1998;39:508–509
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