Shahab Abid ( Department of Medicine, Medical Unit III, Civil Hospital, Karachi. )
Khursheed Memon ( Department of Medicine, Medical Unit III, Civil Hospital, Karachi. )
A 20 years old male presented with a history of developing subcutaneous masses and fever 5 months back, which subsided in 15 days. Four days before admission he again developed fever along with fresh crops of subcutaneous masses, headache and fatigue. On admission the patient was toxic, temperature 39.2°C. Heart rate 120/mm., B.P. 110/80 and there were subcutaneous nodules over the trunk, legs and ann, measuring 2-10 cm with overlying erythematous bluish hue, firm to hard in consistency and slightly tender (Figure 1).
The systemic examination was unremarkable. Investigations showed a haemoglobin of 1 1.4G/dl, total leukocyte count was 8, 700/cumm with a normal differential. ESR was 48 mm/lst hour. Peripheral film for malarial parasites were repeatedly negative. His random blood sugar was 164 mg% and blood urea, creatinine and electrolytes were within normal limits. No growth was obtained on blood cultures. His liver function tests were marginally deranged and blood wasnegative for HBsAg. Serum amylase, ASO titre, C3, C4 and Immunogloblins A, E, G, and M, AMA, ASMA, anti DNA and skin tests for tuberculin and leprosy were negative or within normal limits. X-ray of chest and ultrasOnography of the abdomen were also normal.
Skin biopsy showed findings consistent with Weber-Christian disease or idiopathic panlculitis (Figure 2).
The patient was treated with prednisilone, and had 2 relapses in 3 months, with regression of nodules in second relapse.
Cause of this disease is not known but experimental evidence implies a role for immune mechanism in this syndrome as do the association with connective tissue disease and the abnormal immune function noted in some cases1
When only subcutaneous fat is involved the prognosis is good, it is also related to underlying etiology, some cases recover after few months and permanent remission after 2-5 years is usual. Rarely recurrence may continue for ten years or more but without serious deterioration of generai condition. Reported deaths have been attributable to intercurrent disease exceptionally visceral involvement may be fatal2. Idiopathic paniculitis Weber Christian disease is rare variety of Paniculitis3.
We are thankful to Dr. Hizbullah Sheikh from Aga Khan University Hospital for providing us the photomicrographs.
1. SculIy, R. E., Mark, E.J. and McNeely, B.U. Weekly clinicopathological exercises; case 17-1982. N. Engi. J. Med., 1982; 306:1035.
2. Rook, A., Wi]kaison, D.S. and Ebling, F.J.G. Textbook of dermatology. 3rd ed. Oxford, Blackwell, 1979,p. 1658.
3. Robbins, S.L., Cortran, R.S. and Kumar, V.Pariiculitis (çrythrema nodosum erythema induratum, and Weber Christian disease), in pathologicbasis of disease. 3rd ed. Philadelphia, Saunders,1984,p.1288.