Abstract

Sir, total clearance of skin lesions could be obtained with a 5 months course of chloroquine 250mg daily. Primary biliary cirrhosis is associated with many immunological abnormalities and is therefore widely held to be an autoimmune disorder (1). Among these are chronic thyreoidDISCUSSION itis, rheumatoid arthritis, Sjogren’s syndrome, scleroderma, The coexistence of primary biliary cirrhosis and other autothe CREST syndrome and systemic lupus erythematosus immune disorders is well documented. However, to the best (2, 3). Here, we report a patient who developed discoid lupus of our knowledge this is the Ž rst report of DLE following erythematosus (DLE) during treatment with cyclosporine primary biliary cirrhosis. It is also of considerable interest that 6 years after liver transplantation for primary biliary cirrhosis. these lesions occurred despite immunosuppressive treatment. There are several reports in the literature showing cyclosporine to be poorly eVective in in uencing DLE at a dosage CASE REPORT up to 5.3mg/kg/day (4, 5). Similar to our observation, Di In November 1999, a 50-year-old woman presented with a Lernia & Bisighini described the onset of DLE in a patient 1-month history of sharply marginated bright-red discoid receiving this drug for recalcitrant psoriasis (6). plaques on the nose, right cheek and scalp, causing a 2-cm The fungal metabolite cyclosporine is a potent immunodiameter area of scarring alopecia. No other skin Ž ndings suppressive agent with a relatively selective action on T were present. Apart from photosensitivity, she did not suVer lymphocytes. However, paradoxical eVects on the immune from any other symptoms. A skin biopsy revealed hyperparaksystem by breaking self-tolerance may occur. Administration eratosis and plugging of hair follicles, epidermal atrophy and of this agent can elicit autoimmune diseases in certain animal inŽ ltrating lymphocytes causing an interface dermatitis. models by inhibiting clonal deletion in the thymus with a Immuno uorescence studies showed granular deposits of IgG consequent release of autoreactive T cells (7, 8). at the basement membrane zone (similar to a lupus band). In summary, it is intriguing to speculate that the predisposiThe patient had a 15-year history of primary biliary cirrhosis tion to an autoimmune disease with primary biliary cirrhosis, leading to liver failure and subsequent liver transplantation in on the one hand, and treatment with cyclosporine, on the 1993. Immunosuppressive therapy consisted of anti-thymocyte other, may have contributed to the development of DLE in globulins immediately after transplantation, a short course of this patient. Our observation also demonstrates that treatment glucocorticosteroids and maintenance therapy with cyclosporwith cyclosporine is poorly eVective in DLE. ine, which was given at a dosage of 175mg daily (3mg/kg of body weight) at the time of admission. Minor rises of liver REFERENCES enzymes in a cholestatic pattern suggestive of a recurrence of 1. Kaplan MM. Primary biliary cirrhosis. N Engl J Med 1996; primary biliary cirrhosis in the allograft settled spontaneously 335: 1570–1580. under this therapeutic regimen. Drug-induced hypertension 2. Hall S, Axelsen PH, Larson DE, Bunch TW. Systemic lupus was treated with a combination of lisinopril 20mg and hydroerythematosus developing in patients with primary biliary cirrhosis. chlorothiazide 25mg daily. The only other medications were Ann Intern Med 1984; 100: 388–389. ursodeoxycholic acid 300mg and calcitriol 0.5mg. 3. Nachbar F, Korting HC, HoVman RM, Kollmann M, Meurer M. At the time of skin biopsy, liver function tests showed a Unusual coexistence of systemic lupus erythematosus and primary cholestatic pattern with a three-fold rise of c-glutamyl transbiliary cirrhosis. Dermatology 1994; 188: 313–317. ferase (62 U/l; normal 6–19) and an elevated alkaline phos4. Heule F, van Joost T, Beukers R. Cyclosporine in the treatment of lupus erythematosus. Arch Dermatol 1986; 122: 973–974. phatase (180 U/l; normal 50–155); the anti-mitochondrial 5. Yell JA, Burge SM. Cyclosporin and discoid lupus erythematosus. antibody titre was 1:160. All other routine laboratory investiBr J Dermatol 1994; 131: 132–133. gations were within the normal range; anti-nuclear anti6. Di Lernia V, Bisighini G. Discoid lupus erythematosus during bodies, anti-neutrophil cytoplasmatic and anti-cardiolipin treatment with cyclosporine. Acta Derm Venereol 1996; 76: 87–88. antibodies could not be detected. 7. Sakaguchi N, Sakaguchi S. Causes and mechanism of autoimmune Treatment of DLE consisted of cryotherapy and a steroid disease: cyclosporin A as a probe for the investigation. J Invest cream (with little success) and subsequently hydroxychloroDermatol 1992; 98: 70S–76S. quine 200mg b.i.d. (6mg/ kg of body weight per day) in 8. Chen W, Thoburn C, Hess AD. Characterization of the pathogenic addition to a potent sunscreen. At a follow-up visit after 6 autoreactive T cells in cyclosporine-induced syngeneic graft-versushost disease. J Immunol 1998; 161: 7040–7046. months, the lesions were only in partial remission. However,

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