3 Table 1. Diagnostic criteria for Kawasaki disease 4. Kato H, Akagi T, Sugimura T, Sato N, Hashino K, et al. Kawasaki disease. Coron Artery Dis 1995;6:194-206. lasting for at least 5 days Presence of four of the following five conditions: 5. Newburger JW, Takahashi M, Beiser AS, Burns Je, Bastian J, Chung KJ, et al. A single intravenous infusion of gamma • Changes in peripheral extremities including , i.e. oedema globulin as compared with four infusions in the treatment of and/ or of or feet, and convalescent, i.e acute Kawasaki . N Engl J Med 1991;324:1633-9. membranous, usually periungual . 6. Terai M, Shulman ST. Prevalence of coronary artery • Polymorphous skin , primarily truncal; but non-vesicular abnormalities in Kawasaki disease is highly dependent on • Bilateral painless non-purulent conjunctival injection dose but independent of salicylate dose. • Changes in lips and oral cavity such as erythema and cracking J Pediatr 1997;131:888-93. of lips, strawberry , and diffuse injection of oral and pharyngeal mucosae 7. Committee on , Endocarditis, and Kawasaki • Cervical Disease, Council on Cardiovascular Disease in the Young, American Association. Diagnosis and therapy of Kawasaki disease in children. Circulation 1993;87:1776-80.

or rupture of may occur during the Girish G. Kamath � acute phase of the illness. , , David Kent Louis G. Clearkin endocarditis, valvular insufficiency, heart failure and Department of may also occur. The Arrowe Park Hospital includes , and other , Wirral CH49 5PE, UK

toxic syndrome, , rickettsial disease, Tel +44 (0)151 6785111 juvenile rheumatoid , drug reactions, Fax +44 (0)151 6047152 Stevens-Johnson syndrome, vasculitides and adenoviral . There are no specific diagnostic tests, but the acute phase reactants are usually markedly elevated. A single high dose of intravenous gamma globulin, Sir, 2 g/kg, given over 12 h and commenced before the tenth Keratoconjunctivitis sicca associated with lichen day of the illness, significantly reduces both the sclerosus et atrophicus incidence and severity of coronary artery Many causes are known for dry eye. Among them are abnormalities.5,6 Therapy with high-dose , autoimmune diseases, infectious diseases, tumours, 80-100 mg/kg per day, is also advised during the febrile genetic disorders and response to or stage and is continued at a single antiplatelet dose for 6-8 radiation. We present a case of bilateral weeks or longer if definite coronary vessel abnormalities keratoconjunctivitis sicca associated with lichen sclerosus exist? Careful and serial follow-up evaluations are et atrophicus (LSA).l warranted for children with significant residual coronary sequelae. In summary, Kawasaki disease is a serious and Case report potentially life-threatening condition. Prompt A 58-year-old woman was admitted to our centre recognition and treatment will, in most cases, reduce the complaining of irritation, itching and foreign body morbidity and mortality associated with this disease. sensation in both her eyes. On ocular examination One of the principal criteria for diagnosiS is a - a positive fluorescein staining of the cornea and of the common presentation for many conditions in both the bulbar with rose bengal was observed. A ophthalmic and general practice settings. As the decrease in tear production (1 mm in a Schirmer I test) incidence of Kawasaki disease increases worldwide, and a tear break-up time of less than 10 s was caused by ophthalmologists and all other healthcare professionals a degree of meibomitis and meibomian gland should be particularly aware of the Significance of this inspissation. No other abnormal ocular findings were often innocuous sign. We would therefore recommend a demonstrated. A diagnosis of keratoconjunctivitis sicca detailed systemic examination in any febrile child with was made. , looking in particular at skin, hands and Treatment with tear substitutes was insufficient for feet, lips, oral cavity and lymph nodes to rule out management, and occlusion of the puncta with collagen Kawasaki disease. plugs was performed. Symptoms of dry eye persisted and the patient underwent an uneventful bilateral References tarsorraphy with marked improvement. 1. Taubert KA. Epidemiology of Kawasaki disease in the United Dermatological examination revealed asymptomatic States and worldwide. Prog Pediatr CardioI1997;6:181-5. 2. Dhillon R, Newton L, Rudd PT, Hall SM. Management of porcelain-white papules and plaques, 0.5-10 cm in Kawasaki disease in the British Isles. Arch Dis Child diameter, located on the neck, trunk, hips and eyelids. 1993;69:631-6. Histological findings from one of the plaques showed an 3. Dajani AS, Bisno AL, Chung KJ, Durack DT, Gerber MA, atrophic epidermis with keratotic plugging. The upper Kaplan EL, et al. Diagnostic guidelines for Kawasaki disease: dermis was oedematous with homogenisation- of American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Am J Dis Child collagen fibres and mild perivascular lymphocytic 1990;144:1218-9. infiltrate.

103 The histological picture was consistent with LSA. reported of bilateral central retinal vein occlusions in a Blood tests were within the normal limits. Serological genotypically male individual which may be secondary tests for rheumatoid factor, antinuclear factor, SSA to the use of synthetic sex hormones. (anti-Ro), SSB (anti-La), anti-smooth muscle , anti-mitochondrial antibody and anti-thyroid antibody Case report were all negative. Normal levels of C3 and C4 were found. from a salivary gland did not reveal any We present a case of a 45-year-old transsexual patient on significant pathology that supported the diagnosis of hormone therapy who presented to the Eye Department Sjogren's syndrome. with a left non-ischaemic central retinal vein occlusion (CRVO) reducing her vision to 6/24 (Fig. la). She had been commenced on 25 I-lg of Comment ethinyloestradiol 11 years previously and was converted LSA is an inflammatory disease of unknown cause and to Ovran (250 I-lg of levonorgestrol and 50 I-lg of incompletely characterised pathogenesis. The majority of ethinyloestradiol) for the last 9 years. She also had been patients are women with genital involvement. treated with 50 I-lg of cyproterone acetate on a daily basis Extragenital LSA is most common on the neck and for 11 years. She smoked in excess of 10 cigarettes a day. shoulders and is generally asymptomatic in both sexes. Her blood pressure was 130/80 mmHg on presentation. An association of autoantibodies and autoimmune She was advised to stop smoking and the hormone diseases with LSA has been shown though its significance therapy on the day of her opthalmic examination, which remains unclear? LSA and morphea are unrelated she duly did. No antiplatelet drugs were prescribed. diseases3 based on clinical and histological differences. Six months later she developed a right non-ischaemic Laws and Kalz4 reported the first case of LSA CRVO (Fig. Ib) which reduced her vision to 6/60. involving the eyelids, with no other ocular abnormalities. Investigations including lipids, lupus anticoagulant, 5 Dalziel et a/. reported LSA and morphea involving the face associated with bilateral superior oblique tendon sheath syndrome (Brown syndrome). Our patient suffered from LSA associated with bilateral severe keratoconjunctivitis sicca and eyelid involvement.

References

1. Meffert JJ, Davis BM, Grimwood RE. Lichen sc1erosus. J Am Acad Dermatol 1995;32:393-416. 2. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen sc1erosus et atroplticus and : a study of 350 women. Br J Dermatol 1988;118:41-6. 3. Patterson JA, Ackerman AB. Lichen sc1erosus et atrophicus is not related to morphea. Am J Dermatopathol 1984;6:323-35. 4. Laws HW, Kalz F. Lichen sc1erosus et atrophicus of the eyelid. Can J Ophthalmol 1968;3:39-42. 5. Dalziel K, Reynolds AI, Holt PJ. Lichen sc1erosus et atrophicus with ocular and maxillary complications. Br J Dermatol 1987;116:735-7. (a) R. Rabinowitz 1 G. Rosenthal1 J. Yerushalmy2 T. Lifshitz 1 1 Department of Ophthalmology 2Department of Dermatology Soroka Medical Center Beer Sheva, Israel

R. Rabinowitz MD � Department of Ophthalmology Soroka Medical Center PO Box 151, Beer Sheva 84 101, Israel

Sir,

A transsexual patient with bilateral central retinal vein occlusions Transsexual patients are treated with high levels of

synthetic sex hormones for gender reassignment and are (b) unaware of possible eye complications. A case is Fig. 1. Fundus photographs of (a) the left eye and (b) the right eye.

104