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Proc. roy. Soc. Med. Volume 68 July 1975

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On examination: Twelve mobile non-tender subcutaneous lumps. The skin over each lump was normal with the exception of the lesion below the right eye where there was yellow discoloration. Eyes showed bilateral episcleritis. Nose: appearances were those of an atrophic rhinitis. Other systemic examination was normal. Investigations:, Normal: heemoglobin, full blood count, platelets, blood film, calcium, phosphate, urea, uric acid, cholesterol, triglycerides, lipoprotein electrophoresis, Wassermann reaction, Reiter protein complement fixation test, rapid plasma reagin test, latex screening test for rheumatoid factor, glucose tolerance test, 5 nucleatidase; X-rays of chest, sinuses and skull. Mantoux test with 1/100 old tuberculin negative. No skin nodule was obtained following the injection of Kveim antigen. The patient refused permission for biopsy. Sternal marrow: specimen was mildly hypercellular. Erythroid and myeloid maturation was normal. Plasma cells were morphologically normal and not increased in number. Abnormal: ESR range 40-60 mm in 1 hour (Westergren). Proteins: an abnormal protein band of IgG kappa specificity was detected. Quantitatively this represented 1.4 g/100 ml. A similar band was detected in the urine. Serum enzymes. Alkaline phosphatase 160 (normal range 30-85 miu/ml). LDH 372 (upper limit of normal 300 miu/ml). SGOT 59 (range 10-50 miu/ml). X-rays of hips and spine: mid-dorsal kyphosis with anterior osteophytes visible. Degeneration L4-5 disc. No destructive bone lesions seen. Histology: Lesions from the left side of the neck, from beneath the breast and from the upper part of the back showed very similar appearances. A subcutaneous, nonspecific granulomatous infiltrate is visible; the process is infiltrating both fat and muscle. Giant cells are present and small

~~~~~~~Fig 1 Subcutaneous lump from left side of neck H &E. xlOO0

areas of fibrinoid collagen degeneration can also be seen. Stains for acid-fast bacilli negative. No doubly refractile material detected under polarized light examination. (Fig 1). Comment The diagnosis remains open. The patient remains well and there has been no change in the subcutaneous lesions. It is planned to reassess her paraproteinmmia at six-month intervals.

Sclerosing Lipogranuloma. ? Necrobiosis Lipoidica R E Bowers MD FRCP (Gloucester Royal Hospital, Gloucester) R R, woman aged 55. Shop assistant History: In November 1971, following an attack of boils, a faint trace of glucose was found in the urine but the glucose tolerance test did not show diabetes. In December 1973 she presented with a firm, telangiectatic yellowish infiltrated aIaque which had developed below the left eye over the previous eighteen months. Similar lesions were found on each breast and on her appendicectomy scar. More recently, the right infraorbital area has become affected while that on the left has ulcerated a little (Fig 1). The lesions are becoming more sclerotic. The spleen is now palpable, blood pressure 170/100. Investigations: Biopsy from right iliac fossa lesion showed panniculitis. There were many giant cells some with foamy cytoplasm and several areas of necrobiosis. Abnormal findings included: plasma viscosity raised 2.29 cP (normal less than 1.75 cP), alkaline phosphatase 168 units SGOT 109, ESR 41 mm in 1 hour (Westergren). Glucose

Section of Dermatology

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Fig 1 Sclerosinggranuloma in August 1974

tolerance test 74, 123, 243, 164, 77 mg/100 ml. Glycosuria 0.6 % at 1 hour, 0.5% at 2 hours. Serum proteins, immunoglobulins, cholesterol, and X-ray of chest skull, spine, long bones and pelvis were normal. Hb 8.5 g/100 ml one year ago responded to oral iron and subsequent blood counts have been normal. Comment The raised plasma viscosity, sedimentation rate and liver enzymes remain unexplained.

Acknowledgment: I am grateful for the assistance of members of the Department of Dermatology, Radcliffe Infirmary, Oxford.

Monoclonal Cryoglobuliniemia P P Seah MB MRCP and P R N Kind MSC MRCPath (for M Feiwel FRCP and Leonard Sash MB) (St Charles' Hospital, London WJJ, and St Mary's Hospitals, London) J S, woman aged 47 History: Since 1962 polyarthritis mainly of hands and feet with subsequent cold sensitivity. Ulcer on left leg in 1969. Early in 1973 she developed ulcers on both lower limbs which according to her were 'definitely related to cold'. Livedo reticularis was noted, also hypogammaglobulineemia, mainly low IgG. Subsequently, cryoglobulins were found. Investigations: Hb 11.9 g/100 ml, WBC 6400/mm3. ESR 12-80 mm in 1 hour (Westergren). Rheumatoid arthritis factor and sheep cell agglutination test, antinuclear antibody and other autoantibodies, LE cell, Australia antigen,

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Wassermann reaction and venereal disease research laboratories test all negative. Skin biopsy of left ulcer non-contributory. Immunofluorescence of 'livedo' skin: no IgG. IgM, IgA, C3 or Clq deposits seen. Urine: No Bence-Jones protein. Creatinine clearance normal. Blood urea 39 mg/100 ml. Protein studies: Cryoglobulin precipitates at 28-30°C; immunoelectrophoresis showed monoclonal IgG with lambda light chain; IgG 12 g/l, IgA nil, IgM nil; cryoprecipitates with first (Cl) component of complement. Supernatant (after removal of cryoglobulins): IgG 13.6, IgA 0.68 (low), IgM 0.65 g/l (normal values 6-16, 1.2-4, 0.5-2 g/l). Total protein 67, albumin 40, globulin 27 g/l (normal values 60-80, 35-51, 27-35 g/l). At room temperature: total haemolytic serum complement and immune complexes (anticomplementary) nil; ESR 60 mm in 1 hour (Westergren). At 37°C: total hmmolytic serum complement 6.8 units (low); immune complexes (anticomplementary) present; ESR 80 mm in 1 hour. The IgG in the supematant was almost entirely monoclonal with lambda light chain, like the cryoglobulin. It could be made to precipitate at 28-30°C by addition of more Cl q. Immunoelectrophoresis showed only a very small amount of polyclonal IgG and severe immunoparesis of other immunoglobulins. Protein measurements a year ago showed immunoglobulins below normal levels, the cryoglobulin (paraprotein) being precipitated by normal clotting and separation techniques. The increase in IgG found in the supernatant of the serum separated at 37°C probably indicates a rise in production of the monoclonal paraprotein (including the cryoglobulin) over the last 12 months. Bone marrow 21.6.74: Increased plasmocytes (11 %) suggestive but not diagnostic of myeloma; iron deficient. 16.9.74: Plasmocytes 14%; immature appearance; suggests myeloma. Bone scan: Increased uptake in sternum and left fifth rib. Skeletal survey normal. Comment After a twelve-year history of undiagnosed polyarthritis, the patient has been found to have a paraprotein cryoglobulinemia and bone marrow changes suggestive of myelomatosis. The cryoglobulin is monoclonal IgG and complement fixing. The first component of complement Cl (detected as Clq) appears to be necessary for the formation of the cryoglobulin - hence, removal of the Clq component by column chromatography abolishes the ability of the monoclonal IgG to precipitate in the cold.