EP10 A rare cause of lobular panniculitis. (20th April 2020)

• Record Type: Journal Article
• Authors: Mudawi, Tareg; Amoasii, Constanta; Clewes, Adrian
• Is Part Of: Rheumatology. Volume 59(2020)Supplement 2
• Publication Date: 2020-04-20
• Subjects: Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keaa109.009
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Background: Weber-Christian disease is a rare inflammatory disorder of the subcutaneous fatty tissue. It is characterised by painful relapsing, subcutaneous nodules occurring preferentially at the upper arm, thigh and trunk regions. The disease is often accompanied by recurrent temperatures and constitutional symptoms. Methods: We aimed to increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. Results: A 65 year old female had been investigated thoroughly within a period of 7 years by a number of dermatology and rheumatology departments of North West hospitals. Symptoms started after mastitis with skin rash in form of macular blanching on upper abdomen and episodes of fever ranging between 38 and 40 degree Celsius, fatigability and arthritis. C-reactive protein was 300 mg/l, Erythrocytes Sedimentation Rate 150 mm/H and haemoglobin 95 g/L, positive lupus anticoagulant and negative anti-nuclear antibodies as well as extractable nuclear antibodies and normal complement levels C3 and C4 . Investigations results showed no evidence of infection, malignancy, vasculitis or connective tissue disease. PET scan revealed subcutaneous fat inflammation with increased uptake in mediastinum fat, initial skin biopsy showed lobular panniculitis with mild dermatitis suggestive of lupus profundus. Symptoms and inflammatory markers responded well to steroids however all tried DMARDs (methotrexate, azathoprime, hydroxychlorquine and mycophenolate) as well as dapsone and colchicine were not successful. In addition, 2 doses of rituximab were also ineffective in controlling her disease. Following a required excessive steroid therapy she developed cataract, osteopenia, and diabetes and became cushingoid. Repeated biopsy from the skin showed mixed inflammatory lobular pannicuilitis with neutrophils, lymphocytes and histiocytic. As per Dermatology Multi-Disciplinary Team the diagnosis of Weber-Christian disease was raised. As her condition continued to be very active she was also referred to London, where treatment with anakinra was organised. Conclusion: The diagnosis of Weber-Christian disease depends on the presence of relapsing fever, systemic inflammation and panniculitis and prognosis varies depending on the response to steroids or DMARDs. For diagnosis you need to exclude all likely differential diagnoses of lobular panniculitis, like infections, certain malignancies, alpha-1-antitrypsin deficiency, pancreatitis and systemic lupus erythematosus. In conclusion, the patient has symptoms and signs as well as investigation results in keeping with Weber-Christian disease. Due to the rare nature of this disease the patient went through, multiple rheumatology and dermatology reviews and investigations and treatment trials over a period of 7 years prior to the correct diagnosis being reached. We believe reporting this case would raise awareness and improve on future patients journeys and care. Disclosures: T. mudawi None. C. Amoasii None. A. Clewes None.