Letters to the editor 767

a two year history of recurrent painful violet found.2 Although M has occasion- and rheumatoid arthritis. Arthritis Rheum when induratum 1990;33:431-5. nodular lesions associated with high ally been isolated erythema 6 Parish W E, Rhodes M D. Bacterial antigens levels of RF (latex; range 1048-2000 U/ml). coincides, most authors support its aetio- and aggregated gamma-globulin in the lesions

She denied asthenia, weight loss, fever, and logical role.3 is a form of of nodular vasculitis. BrJr Dermatol 1967; 79: Ann Rheum Dis: first published as 10.1136/ard.52.10.767 on 1 October 1993. Downloaded from had no respiratory or articular complaints. multifactorial lobular that may 131-47. 7 Pacheco A, Mateos P, Medina J, Guisasola L, On physical examination many scattered, coexist with a tuberculous focus (erythema Carrillo F, Perez-Oleiza J. Tuberculosis painful, tender and slightly raised violet induratum of Bazin) or may not (nodular pulmonar y psirpura de Schonlein-Henoch. subcutaneous nodules were found on the vasculitis erythema induratum complex). Rev Clin Esp 1987; 180: 515. Throughout the two year follow up of our 8 Niemi K M, Forstrom L, Hannuksela M, posterior aspects of both calves. The patient Mustakallio K K, Salo 0 P. Nodules on the was afebrile. Results of cardiopulmonary and patient no nodule became ulcerated and the legs. A clinical, histological and immuno- abdomipal explorations were unremarkable. lesions healed with specific chemotherapy. histological study of 82 patients representing White blood cell and platelet count were Rheumatoid factors in low titres are found different types of nodular panniculitis. Acta normal. The haemoglobin was 126 g/l and in a small percentage of young adults. A high Derm Venereol (Stockh) 1977; 57: 145-54. 9 Harahap M. Tuberculosis of the skin. Int J erythrocyte sedimentation rate 18 mm/lst h. prevalence of RF is also found in patients Dermatol 1983; 22: 524-45. Urea nitrogen, creatinine, glucose, bilirubin, with chronic infections, such as , aspartate transaminase, alkaline phosphatase, , and tuberculosis. These observations cholesterol, triglycerides, calcium, and phos- have suggested that extensive or persistent phorus were all normal. A positive purified exposure to antigens and immune complex protein derivative skin test, antinuclear anti- formation induces the synthesis of RF.4 CREST syndrome with bodies, cryoglobulins, and serum comple- M tuberculosis antigens can induce the pro- pericardial but not ment (C3 and C4) were unremarkable. duction of RF, and several reports have Serum RF determined by turbidimetric demonstrated the autoimmunogen capacity peripheral calcinosis immunoassay was 2048 U/ml (Quantex RF of some proteins of this microorganism, Sir: The CREST subgroup of systemic plus latex; WHO units). Rheumatoid factor particularly heat shock protein 60.5 sclerosis is a mild or slowly progressive form determined by haemagglutination on slide Immune complexes seen to have an import- of the disease characterised by calcinosis, (modified Waaler-Rose procedure) was ant pathogenic role in nodular vasculitis.6 Raynaud's phenomenon, oesophageal dys- 256 U/ml (Celarkit AR; Biokit SA, Spain; They have been found in patients with function, sclerodactyly, and telangiectasia.' WHO units). Henoch-Schonlein purpura associated with Anticentromere antibodies detected on the Chest films showed a fibroproductive active tuberculous infection.7 It should be Hep-2 substrate appear highly selective for pattern affecting both upper lung lobes with- pointed out that in our patient autoimmune this group of patients.2 In the Johns Hopkins out paratracheal or perihiliary adenopathies. diseases with increased RF levels, such as series calcinosis was present in all patients Microscopic examination of three sputum rheumatoid arthritis, Sjogren's syndrome, with CREST, being peripheral or around specimens stained by Ziehl-Neelsen's method cryoglobulinaemia, and autoimmune liver joints in the majority.' Patients with CREST disclosed no acid-fast bacilli. A wedge biopsy disease, were excluded. Moreover, after treat- do develop internal organ manifestations but of one skin nodule was performed and patho- ment was started RF progressively fell to usually only in the second and third decades logical examination disclosed a granu- nearly normal values. of their disease.3 We describe a patient with lomatous panniculitis consistent with The persistent negativity of the Mantoux the CREST syndrome who presented with erythema induratum (figure). Ziehl-Neelsen skin test deserves a special mention. It is dyspnoea due to calcific pericarditis without and auramine-rhodamine stains were unlikely that the patient had disseminated peripheral calcinosis. negative. Sputum cultures in L6wenstein- tuberculosis or any other immunosuppressive A 65 year old white female smoker Jensen medium grew M tuberculosis. condition. A negative Mantoux skin test has presented to her general practitioner with Specific daily treatment with rifampicin been described in patients with erythema shortness of breath in August 1989. He 600 mg, isoniazid 300 mg, and pyrazinamide induratum.2 There is no doubt about the detected an expiratory wheeze and prescribed 1500 mg was started and continued for two importance of the skin biopsy as it allows amoxycillin. Her pulse was 160 beats/min, months. Rifampicin and isoniazid was histological demonstration of erythema indu- and she was referred to a cardiologist. She continued for four more months at the same ratum and helps to establish the differential was in atrial flutter with 2:1 block and had dose. The two Mantoux tuberculin skin tests diagnosis with , syphilis, signs of 'mild congestive heart failure'. The http://ard.bmj.com/ (5 tuberculin units of purified protein foreign body granuloma, and skin tuber- jugular venous pressure was raised 9 cm. A derivative) performed while the patient was culosis.8 9 Finally, we strongly believe that chest radiograph showed a cardiothoracic receiving chemotherapy were negative. At the the finding of erythema induratum should ratio of 58% with upper lobe venous diver- end of the first, third, and sixth months prompt a diagnostic search for pulmonary or sion. She was anticoagulated and cardio- of treatment, RF had decreased to 600, 200, extrapulmonary involvement of tuberculous verted but atrial flutter recurred, and her and 44-2 U/ml respectively. The patient infection. ventricular rate was controlled with vera- remained asymptomatic, and skin lesions The authors thank Miss Sonia Galeron for pamil, digoxin, and a diuretic. The patient

healed leaving a pigmented scar. on October 6, 2021 by guest. Protected copyright. assistance in the preparation of this manuscript. had a six year history of Raynaud's phenom- Erythema induratum, included in the tuber- enon and was noted to have facial telangiec- culids, is not a 'true' manifestation of skin NORBERTO GOMEZ RODRIGUEZ tasia and mild sclerodactyly. Further investi- tuberculosis because acid-fast bacilli are not JOSE LUIS FERREIRO SEOANE gation showed a positive anticentromere EMILIA FORMIGO RODRIGUEZ Rheumatology Unit antibody and negative antinuclear and Scl-70 Povisa Medical Center (topoisomerase 1) antibodies. A barium Spain study showed markedly reduced oesophageal ANA DE IA FUENTE BUCETA motility. A diagnosis of CREST syndrome Department ofPathology was made, though no calcinosis had been Povisa Medical Center detected either clinically or radiologically. Spain She gained symptomatic relief from wearing Correspondence to: Dr Norberto G6mez heated gloves and using cisapride, having Rodriguez, Rheumatology Unit, Povisa Medical been intolerant of H2 blockers. Center, C/ Salamanca no 5, 36.211 Vigo, Spain. Although there was improvement in her 1 Feiwel M, Munro D D. Diagnosis and treat- dyspnoea, the jugular venous pressure ment of erythema induratum (Bazin). BMJ3 remained raised. In March 1991 she reported 1965; i: 109-1 1. increasing shortness of breath on exertion 2 Bondi E E, Lazarus G S. Panniculitis. In: and swelling of the legs. Her jugular venous Fitzpatrick T B, Eisen A Z, Wolff K, Freedberg I N, Austen K F, eds. Dermatology pressure was 8 cm raised with a positive in general medicine, 3rd ed. New York: Kussmaul's sign and pulsus paradoxus of 30 McGraw-Hill, 1987: 140-1. mmHg. A lateral chest radiograph showed 3 Hassoun P M, Shepherd K E, Flotte T T, Kazemi H. Erythema induratum and active marked pericardial calcification (figure) that pulmonary tuberculosis. Am Jf Med 1988; 84: had not been apparent on previous postero- 784-5. anterior views. There was no history of tuber- 4 Bartfeld H. Distribution of rheumatoid factor culosis or tuberculous contacts. The possibil- Nodular panniculitis. Some granulomata activity in non-rheumatoid states. Ann NY show caseous central necrosis (haematoxylin Acad Sci 1969; 168: 30-8. ity of an operation to relieve her tamponade and eosin). 5 Rook G, Lydyard P, Stanford J. Mycobacteria was discussed, but the patient refused. 768 Letters to the editor

with bronchodilator treatment. Respiratory P SHELDON Department ofRheumatology function tests showed reduced lung volumes Leicester and an obstructive defect. The peak flow Royal Infirmary

Leicester Ann Rheum Dis: first published as 10.1136/ard.52.10.767 on 1 October 1993. Downloaded from improved from 145 to 200 I/min (predicted 319 1/min) after nebulised salbutamol. The A J McCANCE J D SKEHAN corrected transfer factor was only marginally Departmient ofCardiology reduced, making significant pulmonary Groby Road Hospital vascular disease unlikely. Leicester Our patient's deteriorating exertional Correspondence to: Dr H G Taylor, Department of dyspnoea and increasing oedema in 1991 Medicine, Wanganui Base Hospital, Private Bag, were due to pericardial constriction and once Wanganui, New Zealand. this had been surgically relieved the presence 1 Tan E M, Rodnan G P, Garcia I, Moroi Y, of obstructive airways disease was unmasked. Fritzler M J, Peebles C. Diversity of anti- Pericardial disease occurs in about 50% of nuclear antibodies in progressive systemic patients with systemic sclerosis4 and only sclerosis: anti-centromere antibody and its relationship to CREST syndrome. Arthritis slightly less commonly (38%) in patients with Rheumn 1980; 23: 617-25. CREST.5 Only a third of these patients are 2 Velayos E E, Masi A T, Stevens M B, Shulman symptomatic, however.6 As far as we know, L E. The "CREST" syndrome. Comparison this is the first reported case of calcific with systemic sclerosis (scleroderma). Arch Intern Med 1979; 139: 1240-4. constrictive pericarditis in the CREST syn- 3 Salemi R, Rodnan G P, Leon D F, Shaver J A. drome and is particularly unusual in that our Pulmonary hypertension in the CREST patient had no evidence of peripheral calci- syndrome variant of progressive systemic sclerosis (scleroderma). Ann Intern Med 1977; Chest radiograph showing dense pericardial fication. 86:394-9. calcification on the lateral view. Lung involvement in systemic sclerosis was 4 Botstein G R, LeRoy E C. Primary heart disease recognised shortly after the original descrip- in systemic sclerosis (scleroderma): advances tion of the disease7 and is found with a similar in clinical and pathologic features, patho- genesis, and new therapeutic approaches. Ampi prevalence in patients with CREST.' Heart3r 1981; 102: 913-9. Within three months, however, she Although large airways obstruction in 5 Smith J W, Clements P J, Leviman J, Furst D, underwent cardiac catheterisation with CREST syndrome and systemic sclerosis has Ross M. Echocardiographic features of pro- simultaneous right and left heart pressure been well described, response to broncho- gressive systemic sclerosis (PSS)-correlation measurements which showed the character- with haemodynamic and postmortem studies. dilator treatment is poorly documented.7 A AmJ_7Med 1979; 66: 28-33. istic dip and plateau configuration of con- small number of children with systemic 6 McWhorter J E, Le Roy E C. Pericardial disease strictive pericarditis. A pericardiectomy was sclerosis have had bronchodilator responsive in scleroderma (systemic sclerosis). AmJn_tMed performed and she made an uneventful 1974; 57: 566-75. obstructive airways disease.' Our patient's 7 Owens G R, Follansbee W P. Cardiopulmonary recovery. On returning to full activity she incomplete but appreciable response and manifestations of systemic sclerosis. Chest again complained of shortness of breath on symptomatic improvement suggests that 1987; 91: 118-27. exertion. Physical examination and chest bronchodilators may be helpful in this 8 Owens G R, Fino G F, Herbert D L, et al. radiography showed no evidence of residual Pulmonary function in progressive systemic situation. sclerosis. Comparison of CREST syndrome pericardial constriction. Spirometry, how- variant with diffuse scleroderma. Chest 1983; ever, showed an obstructive picture with a H G TAYLOR 84: 546-50. forced expiratory volume in one second of Departmnent ofMedicine 9 Garty B-Z, Athreya B H, Wilmott R, Scarpa N, Wanganui Base Hospital Doughty R, Douglas S D. Pulmonary func- 0-85 litres and forced vital capacity of 1-6 Wangantui tions in children with progressive systemic litres. Her symptoms improved considerably New Zealand sclerosis. Pediatrics 1991; 88: 1161-7. http://ard.bmj.com/ on October 6, 2021 by guest. Protected copyright.