anticardiolipin antibodies and lupus anticoagulant syndrome' ... syndrome. Case report. A 25-year-old female ... Association-(Tan et al. 1982) criteria for systemic.
Journal ofthe Royal Society ofMedicine Volume 78 August 1985 677
Systemic lupus erythematosus with anticardiolipin antibodies and lupus anticoagulant syndrome' Ali S M Jawad DCH MRCP H Berry DM FRCP Department of Rheumatology & Rehabilitation King's College Hospital, London SE5 9RS A strong relationship between anticardiolipin antibodies, the lupus anticoagulant and thrombosis has recently been demonstrated in patients with systemic lupus erythematosus (SLE) and 'lupuslike' disorders (Harris et al. 1983). We describe a patient with SLE with strongly positive anticardiolipin antibodies and the lupus anticoagu1ent syndrome.
were also involved. There was an effusion in the left knee and the metatarsophalangeal joints on both sides were tender. Urine examination was normal. Investigations were as follows: haemoglobin 12.8 g/100 ml; white cell count 4900/mm3; platelets 122 000/mm3. X-rays of hands and feet failed to show any erosions. Rheumatoid factor and antinuclear factor were both negative. Extractable nuclear antigen was positive. Double-stranded DNA binding 20.9 (upper normal: 14); antimitochondrial antibodies positive (1/160). Lupus anticoagulant (using the technique described by Exner et al. 1978) was negative, and anticardiolipin antibodies were strongly positive. Discussion
Our patient fulfils the American Rheumatism Association -(Tan et al. 1982) criteria for systemic lupus erythematosus (SLE). In addition, she had recurrent deep vein thrombosis, pulmonary embolism, persistent thrombocytopenia, one unexplained abortion and strongly positive anticardiolipin antibodies. Harris et al. (1983) developed a sensitive solid phase radioimmunoassay for anticardiolipin antibodies some 200-400 times more sensitive than the precipitation method used in the VDRL test. High levels of anticardiolipin antibodies of at least one immunoglobulin class were found in 61 % of serum samples from patients with SLE. There was a strong correlation between raised anticardiolipin levels and the lupus anticoagulant, venous and arterial thrombosis and thrombocytopenia, but no correlation with anti-DNA antibody levels. Of the 15 patients with the highest anticardiolipin titre, 6 had a history of venous thrombosis, 5 developed thrombosis, 5 thrombocytopenia and 2 pulmonary hypertension and multiple abortions. It was concluded that the solid phase radioimmunoassay appeared to have a predictive value for thrombosis in SLE and related disorders. Several other studies have shown a relationship between lupus anticoagulant and venous and arterial thrombosis (Bowie et al. 1963, Mueh et al. 1980, Landi et al. 1983, Boey et al. 1983). The association between the lupus anticoagulant and repeated unexplained intrauterine fetal death is well documented (Nilsson et al. 1975, Solier & Boffa 1980, Firkin et al. 1980, Carreras et al. 198 lb, Lubbe et al. 1983). The lupus anticoagulant is an acquired immunoglobulin, IgG or IgM or even both in the same patient, and is a prothrombinase complex inhibitor, i.e. it inhibits activated factor X, factor V, phospholipid and calcium ions, it therefore affects both intrinsic and extrinsic clotting pathways, hence causing slight prolongation of the pro1Case presented to Section of Rheumatology & Rehabili- tnrombin time and kaoiin partial thromboplastin time (Green et al. 1983). tation, 10 October 1984. Accepted 21 March 1985
Case report A 25-year-old female Caucasian was seen in the rheumatology clinic in October 1983 because of pain and stiffness in her hands, feet and knees. Since January 1978 she had been treated by her general practitioner for arthralgia in her hands, feet and knees accompanied by morning stiffness lasting 2-3 hours. Non-steroidal anti-inflammatory drugs were prescribed and proved effective in relieving her symptoms. In September 1981 she developed deep vein thrombosis in the left leg which was confirmed by a venogram. She was treated as an inpatient with heparin and later warfarin. It was noted that her platelet count was 90 000/mm3. Two months later, whilst still on warfarin, another attack of deep vein thrombosis was followed by two attacks of pulmonary embolism, confirmed with a ventilationperfusion scan. Again her platelet count was noted to be low at 148 000/mm3. Her autoantibody screen was negative. In April 1982 the warfarin was stopped because she wanted to get pregnant; two months later she conceived and her arthralgia resolved completely. When she was 25 weeks pregnant, it was discovered during a routine scan that the foetus in utero was dead and so the pregnancy was terminated. A few days later her arthralgia recurred. In June 1983, she developed sudden shortness of breath with chest pain and palpitations and was admitted to hospital again. A ventilationperfusion scan confirmed the diagnosis of pulmonary embolism. She was treated with heparin and later warfarin. Again her platelet count was 122 000/mm3. On physical examination, she had a facial butterfly rash and mild symmetrical polyarthritis affecting the metacarpophalangeal joints and some of the proximal interphalangeal joints; both wrists
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( 1985 The Royal Society of Medicine
678 Journal ofthe Royal Society ofMedicine Volume 78 August 1985 Paradoxically, however, it appears to have a hypercoagulable effect. There are at least two explanations for the latter effect. The lupus anticoagulant reacts with platelet wall phospholipids (platelet factor 3) (Thiagarajan et al. 1980), damaging them and increasing their adhesiveness and thereby initiating thrombosis. This may also explain the accompanying thrombocytopenia. Carreras et al. (1981a) found that the lupus anticoagulant inhibited the production of prostacyclin in the pregnant uterus, possibly by interfering with the release of arachidonic acid. This leads to placental thrombosis and hence intrauterine death. We conclude that a search for anticardiolipin antibodies and for lupus anticoagulant is indicated in patients with venous or arterial thrombosis, pulmonary hypertension (Asherson et al. 1983), thrombocytopenia or repeated intrauterine fetal death even in the absence of SLE or 'lupus-like' disorders (Lancet 1984). Acknowledgment: We are grateful to Dr G R V Hughes and his colleagues at the Hammersmith Hospital, London, for measuring the anticardiolipin antibodies in this patient.
Rhinophyma' J 0 Roberts MA FRCS C M Ward BSC FRCS Department of Plastic Surgery West Middlesex University Hospital, Isleworth
Rhinophyma is a relatively common acquired deformity of the nose, -the treatment of which has caused much dispute. A case is reported of a huge rhinophyma which was treated successfully by surgical shaving and spontaneous re-epithelialization. Case report A 73-year-old man presented with a huge rhinophyma (Figure IA) which had started as a gradually increasing thickening of the nasal skin two years previously. An enormous increase in size over the six months prior to presentation had 'Case presented to Sections of Dermatology and Plastic Surgery, 16 February 1984. Accepted 21 March 1985
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References Asherson R A, Mackworth-Young C G, Boey M L et al. (1983) British Medical Journal 287, 1024-1025. Boey M L, Colaco C B, Gharavi A E, Elkon K B, Loizou S & Hughes G R V (1983) British Medical Journal 287, 1021-1023 Bowie E J W, Thompson J H, Pascuzzi C A & Owen C A (1963) Journal of Clinical Investigation 62, 416-430 Carreras L 0, Machin S J, Deman R et al. (1981 a) Lancet i, 224-246 Carreras L 0, Vennylen J, Spitz B & Van Assche A (1981b) British Journal of Obstetrics and Gynaecology 88, 890-894 Exner T, Rickard K A & Kronenberg H (1978) British Journal ofHaematology 40, 143-151 Firkin B G, Howard M A & Radford N (1980) Lancet ii, 336 Green D, Hougie C, Kazmier F J et al. (1983) Thrombosis and Haemostasis 49, 144-146 Harris E N, Gharavi A E, Boey M L et al. (1983) Lancet ii, 1211-1214 Lancet (1984) i, 157-1158 Landi G, Calioni M V, Sabbadini M G, Mannucci P M & Candelisle L (1983) Stroke 14, 377-379 Lubbe W F, Butler W S, Palmer S J & Liggins G C
(1983) Lancet i, 1361-1363 Mueb J R, Herbst K D & Rapaport S I (1980) Annals ofInternal Medicine 92, 156-159 Nilsson I M, Astedt B, Hedner U & Berezin D (1975) Acta Medica Scandinavia 197, 153-159 Solier R P & Boffa M C (1980) Nouvelle Presse Medicale (Paris) 9, 244-246 Tan E M, Cohen A S, Fries J F et al. (1982) Arthritis and Rheumatism 25, 1271-1277 Thiagarajan P, Shapiro S S & De Marco I (1980) Journal of Clinical Investigation 66, 397-405
caused such cosmetic embarrassment that the patient was living in total isolation. In addition the heavy, pendulous growth acted as a valve, obstructing the nares and causing breathing difficulty (Figure 1B). Under general anaesthetic the nose was injected with local anaesthetic containing adrenaline to reduce bleeding. The growth was then shaved off using a scalpel. At the end of the procedure haemostatic foam was applied to encourage haemostasis and provide a dressing. This separated spontaneously at six days leaving a clean, dry wound (Figure lc) which epithelialized comupletely within two weeks. The final result was both functionally and cosmetically acceptable to the patient (Figure ID). Discussion
Rhinophyriia is a tlypQrzi-ophy of the skin ot thL lower half of the nose which develops over a period of years. The resulting lesion varies in size from mild localized or generalized overgrowth to a large, bulbous and discoloured mass. © 1985 The Royal Society of Medicine
