Kaposi's sarcoma in an elderly patient with rheumatoid arthritis after intra-articular corticosteroids

S. P. Burnet and J. D. McNeil

Department of Medicine, Modbury Public Hospital, Smart Road, Modbury, South Australia 5092, Australia

SIR, Kaposi's sarcoma (KS) is an uncommon dermatological malignancy that has previously been associated with rheumatoid arthritis (RA) [110]. Vincent et al. [11] recently reported in this journal two cases of KS following low-dose corticosteroid treatment for other rheumatological disorders. Corticosteroid treatment, enteral and parenteral, has been suggested as an aetiological factor in previous case reports of RA and KS [1, 510]. We describe a case of these two conditions coincident in an elderly patient treated with recent intra-articular corticosteroids.

A 92-yr-old caucasian female hostel resident presented to our hospital with an acute onset of a dense left hemiparesis. A CT scan of her head revealed evidence of an ischaemic event in the right temporal region. There was no haemorrhage, collection or mass. A diagnosis of an ischaemic right cerebrovascular accident was made and her hemiparesis was treated conservatively. Little functional recovery returned and she was transferred to a nursing home 5 weeks after presentation.

Her significant medical history included paroxysmal atrial fibrillation, congestive heart failure and seropositive RA. Her medications on admission included digoxin, frusemide, potassium supplements, ranitidine, verapamil, paracetamol and ibuprofen as required. Her general practitioner had managed her RA for at least 40 yr. She had had monthly intramuscular gold for approximately 15 yr but had ceased this 5 yr prior to her current admission. She had been on no other disease-modifying anti-rheumatic agents. There was no history of oral corticosteroid use. Intra-articular corticosteroids (methylprednisolone 40 mg) had been given into one knee three times in the year prior to presentation and oral analgesics were used as required.

On day six of the admission, staff reported new papular, darkened and ulcerated lesions on the posterior aspect of both calves with a small amount of haemoserous ooze. Over the next 2 weeks the lesions persisted and extended laterally and anteriorly over both lower limbs (Fig. 1Go).



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FIG. 1. Photograph of anterior aspect of right lower limb.

 
Investigations at this time included normal electrolytes, creatinine, complement studies, haemoglobin, platelet count, and white cell count was minimally elevated. Liver function was normal with the albumin just below the normal range. c-ANCA (cytoplasmic pattern antineutrophil cytoplasmic antibodies) was negative and there was a low titre of antinuclear antibodies. A urine specimen revealed a trace of blood and protein but no casts or infection. Erythrocyte sedimentation rate was 103 mm/h and C-reactive protein 128 mg/l (normally <9 mg/l). An HIV test was negative by enzyme immunoassay. A bilateral skin biopsy was performed and Kaposi's sarcoma was diagnosed histologically (Fig. 2Go). A conservative approach was adopted with respect to management of the KS, given the clinical scenario.



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FIG. 2. Slide of skin biopsy (x200) demonstrating a mixed proliferation of irregular slit-like vascular channels associated with pleiomorphic spindle cells, a low-grade lymphocytic inflammatory infiltrate, extravasated erythrocytes and brown haemosiderin pigment consistent with a diagnosis of KS.

 
KS is an uncommon dermatological malignancy composed of proliferating capillary vessels and perivascular connective-tissue cells. In addition to its epidemic occurrence in patients with the acquired immune deficiency syndrome, KS has been associated with solid organ transplants, other malignancies, autoimmune disorders and immunosuppressive therapy [1].

Previous authors [1, 510] have implicated oral corticosteroids, intra-articular corticosteroids and even captopril in the onset of KS in patients with RA. Casoli and Tumiati [1], in their review of the literature of nine patients with RA and KS, however, believe that the risk of an individual patient with RA developing KS is small, and that KS represents the result of a complex interaction between geographic, genetic, environmental and immunological factors. Vincent et al. [11] believe the role of corticosteroids in the development of KS is two-fold, firstly by up-regulation of Kaposi cell proliferation and secondly by activation of human herpesvirus-8 (a virus isolated from all types of KS lesions).

Our patient was in her nineties, had had a recent catastrophic cerebral event, had not taken oral corticosteroids, had not been on gold for 5 yr, had not taken captopril but had received three corticosteroid joint injections in the past year. We feel that there is a relationship between RA and the development of KS in our case and it is interesting to note that this occurred after three recent corticosteroid injections and became apparent whilst an in-patient following a large intracerebral event. We feel that this lady's age, RA, recent parenteral corticosteroid treatment and large cerebrovascular accident all played a role in the subsequent development of KS.

Our case also highlights the usefulness of skin biopsy in the setting of a patient with long-standing RA and new bilateral leg ulcers. Vasculitis must be ruled out in this context as management for this would include high-dose corticosteroids.

Notes

Correspondence to: J. McNeil. Back

References

  1. Casoli P, Tumiati B. Rheumatoid arthritis, corticosteroid therapy and Kaposi's sarcoma: a coincidence? A case and review of the literature. Clin Rheumatol1992;11:432–5.[ISI][Medline]
  2. Gange R, Wilson J. Kaposi's sarcoma and immunosuppressive therapy: an appraisal. Clin Exp Dermatol1978;3:135–46.[ISI][Medline]
  3. Klepp O, Dahl O, Stenwig J. Association of Kaposi's sarcoma and prior immunosuppressive therapy: a 5-year material of Kaposi's sarcoma in Norway. Cancer1978;42:2626–30.[ISI][Medline]
  4. Piette A, Prier A, Camus J et al. Association polyartérite rhumatoide–maladie de Kaposi. A propos d'une observation. Rev Rhum1981;48:735–7.[ISI][Medline]
  5. Valsecchi R, Perego G, Rossi A et al. Kaposi's sarcoma complicating steroid therapy for rheumatoid arthritis. Ann Ital Dermatol Clin Spe1984;38:413–8.
  6. Schottstaedt MW, Hurd ER, Stone MJ. Kaposi's sarcoma in rheumatoid arthritis. Am J Med1987;82:1021–6.[ISI][Medline]
  7. Weiner S, Noritake D, Paulus H. Kaposi's sarcoma in a patient with rheumatoid arthritis and polymyositis treated with corticosteroids. West J Med1988;148:702–3.[ISI][Medline]
  8. Labre J, Nicolas J, Collet P, Labre B, Llorca G. Kaposi's sarcoma in a patient with rheumatoid arthritis. Possible responsibility of captopril in the development of lesions. J Rheumatol1991;18:476–7.[ISI][Medline]
  9. Azais I, Lambert deCursay G, Deblais F et al. Association of rheumatoid arthritis and Kaposi's disease. A propos of a case arising after intraarticular corticotherapy (in French). Rev Rhum1993;60:240–4.[ISI]
  10. Hortelano Martinez E, Calvo Catala J, Lifiana Santafe JJ, Gonzalez-Cruz MI, Soler Ross JJ, Fontbuena Moreno M. Visceral and cutaneous Kaposi's sarcoma in a patient with rheumatoid arthritis under steroid treatment. Ann Med Intern1994;11:77–9.
  11. Vincent T, Moss K, Colaco B, Venables P. Kaposi's sarcoma in two patients following low-dose corticosteroid treatment for rheumatological disease. Rheumatology2000;39:1294–6.[Free Full Text]
Accepted 14 June 2001