Poncet\'s disease. A case report

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SA MEDIESE TYDSKRIF DEEL 65 9 JUNIE 1984

Poncet's disease A case report A. BENATAR,

I. D. HILL,

M. D. BOWIE

Summary A patient with tuberculosis and polyarthritis is described. The investigations and rapid resolution of the arthritis in response to therapy suggested the diagnosis of Poncet's disease. The distinction between Poncefs disease and tuberculous arthritis is prognostically important S AIr Med J 1984; 15: 930-931.

Tuberculous arthritis, the commonest form of tuberculous joint disease, results from infection of the synovium or adjacent bone by Mycobaccerium cuberculosis. 1 It usually involves only one joint and is destructive. In contrast, Poncet's disease or tuberculous rheumatism is a polyarthritis associated with visceral tuberculosis. 2 It appears to be a hypersensitivity reaction to tuberculoprotein as there is no evidence of bacterial infection of the joints or their surrounding structures. 2 While this form of arthritis is far less common, its recognition is imponant as it carries a favourable prognosis and responds rapidly to treatment with antituberculosis drugs. This repon describes a child with tuberculosis and polyarthritis thought to be an example of Poncet's disease.

Case report The patient, a 4-year-old Black girl from Transkei, was admitted to the Red Cross War Memorial Children's Hospital, Cape Town, with a I-day history of pain and swelling of the right elbow joint. Two months before admission she had developed a swelling in the right cervical region and painful nodular lesions of both shins. Her maternal aunt in Transkei was on treatment for pulmonary tuberculosis. Immunizations were 4lcomplete although she had received BCG at the age of 3 years. On examination she was afebrile and thin, with a height and weight below 80% of those expected for her age. She had numerous tender nodules oferythema nodosum over the anterior aspect ofboth legs. The cervical lymph nodes were enlarged, firm and matted, those on the right being larger than the left, and there was prominent enlargement of the epitrochlear lymph nodes. The right elbow and both knees were swollen and there were obvious effusions in both knee joints. Painful limitation of

Department of Paediatrics and Child Health, Institute of Child Health, Red Cross War Memorial Children's Hospital, Cape Town A. BENATAR, M.B. CH.B., F.C.P. (S.A.), D.C.H. 1. D. HILL, M.D., F.c.P. (S.A.), D.C.H. M. D. BOWIE, M.D., B.sc., F.R.C.P., D.C.H.

movement of the affected joints with redness and evidence of tenderness to palpation was present. . Special investigations included a chest radiograph which showed bilateral hilar adenopathy with patchy".consolidation of both lower lobes. No bony abnormality was seen on radiographs of the affected joints. A Mantoux test using only 1 tuberculin unit of purified protein derivative (PPD) produced an area of induration measuring 15 x 20 mm. The erythrocyte sedimentation rate was 95 mm/1st h and the haemoglobin value was normal. The white cell count was 11,5 x 109/1 with polymorphonuclear . predominance. The latex fixation test, anti-DNA antibody test, antistreptolysin-a test and blood culture were all negative and LE cells were absent. Excision biopsy of a cervical lymph node revealed histological findings typical ofa tuberculous granuloma with caseation, Langhans' giant cells, epithelioid cells and fibrosis. Biopsy of one of the nodules on the skin demonstrated a granuloma in the deep dermis with inftltration by epithelioid cells and Langhans' giant cells. Synovial biopsy of a knee joint revealed a mild chronic inflammatory cell infiltrate but no histological features characteristic of tuberculosis. The patient was treated with rifampicin, isoniazid, pyrazinamide and ethambutol. Within 1 week ofcommencing therapy the lesions oferythema nodosum had faded and by 3 weeks the joint symptoms had resolved completely. The patient's further course was uncomplicated and at the time of discharge she was well. Treatment was continued on an outpatient basis.

Discussion This patient had radiographic features compatible with pulmonary tuberculosis together with cervical adenopathy, erythema nodosum and polyanhritis. Confirmation of tuberculous disease was obtained on histological examination of an excised lymph node. The joint manifestations and erythema nodosum appeared to be related to the tuberculosis. Rheumatoid arthritis and connective tissue disorders were considered unlikely in view of the negative laboratory investigations and the rapid resolution following the introduction of antituberculosis drugs. The simultaneous involvement of more than one joint, the clinical course and the lack ofhistological evidence oftuberculosis of the synovium all favour the diagnosis of Poncet's arthritis or tuberculous rheumatism. Although recognized by F reIich workers in the mid-19th century,3 the association between tuberculosis and polyarthritis was first fully described by Antonin Poncet4 in 1897. He initially reponed the condition in a l5-year-old boy with recurrent attacks of polyarthritis associated with a suppurating tuberculous hip, but subsequently reponed many more cases and reviewed the evidence for the entity of tuberculous rheumatism. 5-7 Clinically, the polyarthritis may occur as the first manifestation of tuberculous infection or present during the course of oven non-articular tuberculosis. 7 While it appears. to be uncommon, the distinction between Poncet's disease and tuberculous arthritis is prognostically important. The former is associated with an

SA MEDICAL JOURNAL VOLUME 65 9 JUNE 1984

excellent prognosis with rapid resolution on commencing therapy and no sequelae. The latter has a protracted course and is often associated with joint destruction and long-term sequelae. l Little is known of the pathogenesis of Poncet's disease, but current opinion favours an immunological reaction. 8 The condition may be regarded as having the same clinical significance as phlyctenular conjunctivitis, erythema nodosum and tuberculides. All appear to indicate a high degree of sensitivity to tuberculoprotein9- 11 and should alert the attending physician to exercising extreme caution in the use ofskin tests for tuberculosis. It is currently recommended that they be avoided for fear of producing local ulceration. If a Mantoux test is performed then a much reduced dose of PPD should be administered. Even a low dose of PPD may produce vesiculation; should this occur it is advisable to apply a steroid-eontaining cream to the site immediately to prevent the development of ulceration.

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REFERENCES 1. Ansell B. Rheumaric Dis01'dlrJ in Childhood. London: Bunerworth, 1980: 27-30. 2. !sues AJ, Sturroek RD. Poncet's disease - fact or fiction? A re-appraisal of tuberculous rheumatism. Tubercle 1974; 55: 135-142. 3. Brav EA, Hench PS. Tuberculous rheumatism.J BoneJoinr S"rg(Br) 1934; 16, 839-866. 4. Poncet A. Polyarthrite tuberculeuse simu1ant des lesions rhumatismales chroniques dHormantes. Gaz H6p (Paris) 1897; 70: 1219. 5. Poncet A. Rhumatisme tuberculeux anbylosant. BIIlI M.... Sac Mid HOp Paris 1903; 20: 841-849. 6. Poncet A, Leriche R. Le Rhumarimrt Tubercrdtur. Paris: Doin, 1909. 7. Poncet A. Rhumatisme tuberculeux abarticulaire. Lyon Mid 1902; 99: 65-72. 8. Jacquelin A, Gratay A, Chiron JP, Olivo H, Schwaru B, Schwaru D. Essai de demembrement des rhumatismes tuberculeux. S.... H6p Paris 1965; 41, 3165-3174. 9. Motley D. Paediatric Priorities in rlre DtfJeloping WO1'ld. London: Bunerworths, 1970: 260-261. 10. Harley RD, Martyn LJ. Abnormalities of the cornea. In: Vaughan Vc, McKay RJ, Behrman RE, eds. Nelson Tex:rboolt of Pediarrics. Philadelphia: WB Saunders, 1979: 1969. I!. Schaller JG. Erythema nodosum. In: Vaughan VC, McKay RJ, Behrman RE, eds. Nelson Texrbook of Pediarrics. Philadelphia: WB Saunders, 1979: 679.

• Amyloid gOItre A case report R. L. KNEEBONE,

H. GREEFF,

AYLWYN MANNELL

Case report

Amyloid goitre can be defmed as a clinically detectable enlargement of the thyroid gland due to the deposition of amyloid. Microscopic deposits of amyloid are commonly found in the thyroid in cases of generalized amyloidosis,l but true amyloid goitre is rare. In 1972 James 2 noted that 50 cases had been reported in the English literature, and he described a further 6 cases from Uganda. By 1982 Amado et al. 3 had identified a total of 80 cases in the world literature. In this article an amyloid goitre with features similar to those of a malignant thyroid tumour is described.

Department of Surgery, Baragwanath Hospital and University of the Witwatersrand, and Department of Anatomical Pathology, South Mrican Institute for Medical Research, Johannesburg R. L. KNEEBONE, M.B. CH.B., Registrar in General Surgery H. GREEFF, M.B. CH.B., Registrar in Histopathology AYLWYN MANNELL, F.R.A.C.S., F.R.C.S., Principal Surgeon

In July 1982 a 46-year-old Black man was admitted to Baragwanath Hospital, Johannesburg, with a 3-month history of an enlarging mass in the neck, vague abdominal discomfort, and recurrent epistaxes. The patient was receiving treatment for hypertension. He had a past history of heavy drinking but had abstained from alcohol since 1974. There w-as no history of tuberculosis, bronchiectasis, renal disease or any other chronic illness. . Systematic questioning elicited complaints of orthopnoea, exertional dyspnoea and ankle oedema. There were no symptoms ofthyroid disease. On physical examination the patient was seen to be well-nourished, with a regular pulse rate of 90/min and a blood pressure of 120/80 mmHg. There was evidence of mild congestive cardiac failure with cardiomegaly, bilateral ankle oedema, an elevated jugular venous pressure and bilateral basal crepitations. The hard, smooth liver was palpable 10 cm below the costal margin. The thyroid gland was diffusely enlarged and indurated with a firm, non-tender, mobile lymph node in the right supraclavicular fossa. Examination of the ears, nose and throat revealed no abnormalities. Laboratory investigations showed a haemoglobin value of 10 g/d1, a white cell count of 11,2 x 109/1 and a random blood sugar level of 4 mmol/l. The serum urea and electrolyte levels were normal. Liver function tests revealed a total protein concentration of63,9 gIl with a reversed albumin!globulin ratio (albumin 25,5 g/l, globulin 38,4 g/l). The liver enzyme levels were raised, with an alkaline phosphatase level of 470 UIl (normal 116 - 283 UIl), lactic dehydrogenase level of 490 UIl (normal 230 - 462 U/l), and a gamma-glutamyl transpeptidase level of 248 U/l (normal 10 - 50 U/l). Thyroid function studies were carried out and the patient was found to be biochemically euthyroid, with a free serum thyroxine level of 17,0 pmol/l (normal 10 - 25 pmol/l),