A child with panniculitis and microhaematuria/proteinuria--an unusual presentation of p-ANCA-positive vasculitis

This 12-year-old girl was first referred to the paediatric nephrological outpatient clinic in January 1994, for the investigation of acute renal failure. The patient is the older child (she has a younger healthy brother) of Introduction non-consanguineous parents of Iraqi origin. Her maternal grandmother is on maintenance haemodialysis due to end-stage renal disease of unknown Panniculitis is defined as inflammation of the subcutaaetiology. neous fat. It may be primary (without an identifiable In August 1992 the patient was treated by her local cause) or secondary [1]. The secondary causes of paediatrician for what was diagnosed as cellulitis of panniculitis include those associated with infection, an ear lobe. During this episode, she was discovered pancreatitis, immunodeficiency, haematological maligto have haematuria and proteinuria (on a dipstick nancies, and connective tissue diseases. Erythema examination). An ultrasound of the kidneys and intranodosa is probably the commonest and best-known venous pyelogram were performed, showing normal form of panniculitis [1]. Other forms are less often seen and recognized, usually leading to a considerable appearance. delay in establishing the precise underlying diagnosis. In September 1993, the patient was diagnosed as An adequate skin biopsy (wedge rather than punch) is suffering from ‘erysipelas’ of the left leg, which, howessential in order to define fully the important histopaever, did not respond to methicillin. Laboratory data thological features. These include the exact nature of at the time showed an erythrocyte sedimentation rate the cellular infiltrate, the presence of vasculitis, and (ESR) of 12 mm in the first hour (Westergren), serum the predominant location of the inflammation, either haemoglobin 12.3 g/dl and a blood sugar level of septal or lobular. 90 mg/dl. Rheumatoid factor, C-reactive protein and In this report, we present a 12.5-year-old girl who antinuclear factor were negative. Antistreptolysin O developed rapidly progressive glomerulonephritis titre was 15400 Todd units. In December 1993, she (RPGN), secondary to pANCA-positive vasculitis. was admitted to the orthopaedic department upon Among her initial manifestations the most prominent presenting with a non-tender subcutaneous mass was a panniculitis presenting in a distinctly unusual (3×4×6 cm) of hard consistency located on the medial manner, namely, that of a mass on the inner aspect of aspect of her left thigh. There was associated weight the left thigh. As a result, the patient was originally loss and fever. No regional or generalized lymphadenoinvestigated for a possible malignancy. Definitive diapathy was evident. The patient’s ESR had increased gnosis was delayed until the demonstration of a pannicto 90 mm/h. Serum haemoglobin was 10.1 g/dl, leukoulitis with vasculitis on a skin biopsy and a crescentic cytes 10 300/ml with a normal differential count, plateglomerulonephritis on a renal biopsy. lets 363 000/ml, urea 40 mg/dl, creatinine 1.2 mg/dl, Immunosuppressive treatment led to a rapid and comalbumin 3.6 g/dl, and cholesterol 172 mg/dl. plete disappearance of the panniculitic process. Renal Urinanalysis revealed persistent haematuria and profunction, however, after an initial partial response, is teinuria (not quantitated at this stage). An X-ray of gradually deteriorating. the thigh showed normal bone architecture. Ultrasound examination of the upper abdomen was without pathology. Computerized tomography of the chest showed Correspondence and offprint requests to: Prof. J. Bernheim, Dept. of Nephrology, Meir Hospital, Kfar Saba, Israel. increased interstitial lung markings. A surgical biopsy