British Journal of Dermatology 2001; 144: 594±596. Lichen myxoedematosus with associated cardiac abnormalities R.MORRIS-JONES, R.C.D.STAUGHTON,* M.WALKER,² D.J.SHERIDAN,³ K.RAJAPPAN,³ J.LEONARD AND C.HARDMAN Department of Dermatology, St Mary's Hospital, London W2 1NY, U.K. Departments of *Dermatology, ²Histopathology and ³Cardiology, Chelsea and Westminster Hospital, London SW10 9NN, U.K. Accepted for publication 14 October 2000 Summary We describe a 42-year-old woman who developed lichen myxoedematosus. Twenty years after the onset of the disease she became breathless and hypertensive, and an echocardiogram showed a mass on the mitral valve, which was thought to be a mucin deposit. Her hypertension was resistant to treatment with combination antihypertensives. To our knowledge, this is the first report to link lichen myxoedematosus with a valvular mucinous mass. This case also demonstrates the slow clinical progression of the disease over 20 years. Key words: heart valves, lichen myxoedematosus, scleromyxoedema Lichen myxoedematosus is a rare disorder of unknown aetiology characterized by proliferation of fibroblasts and excessive deposition of acid mucopolysaccharides in the upper dermis. Clinical features consist of groups of lichenoid papules and extensive thickening of the skin. There is frequently an associated paraproteinaemia of IgG class l . Case report A 42-year-old Jamaican woman presented initially in 1979 with asymptomatic discrete whitish papules and nodules on the face, ears, upper chest, thighs and the dorsum of both hands (Fig. 1). She was otherwise fit and well. The disfiguring nature of these lesions was extremely distressing to her, and she had become a recluse. A skin biopsy was taken from the right hand and Alcian blue stain showed mucin deposits consistent with the clinical diagnosis of lichen myxoedematosus. She was started on prednisolone 20 mg daily for 6 weeks but there was continued worsening of her rash. Several nodules on the bridge of the nose were excised but this resulted in unacceptable scarring. Dermabrasion to the nose was then attempted with a carbon dioxide laser, with initial cosmetic benefit, which was not sustained. At this time she was lost to follow-up, but presented 20 years later with progression of the disease with new papules and nodules (Fig. 2). There was exaggera- tion of the facial ridges, her skin had became lichenified and thickened (Fig. 3), she had microstomia and limited finger flexion and extension. She complained of exertional dyspnoea. Her pulse was 70 min 21 and regular, blood pressure 165/110 mmHg, the jugular venous pressure was not raised, and there was a positional dependent mitral murmur heard in the left lateral position. Fundoscopy revealed a modest degree of vascular change with sheathing and crystalline- looking lesions associated with vessels. Blood tests revealed a raised creatinine phosphokinase (100% CPK-MM). A paraproteinaemia of IgG l class was detected and total serum protein was mildly raised at 85 g L 21 (normal 60±80). Chest X-ray showed cardio- megaly and mild heart failure, and electrocardiography showed sinus rhythm with a left bundle branch block. A transthoracic echocardiogram showed mild con- centric left ventricular hypertrophy with mild aortic and mitral valve regurgitation. The most striking feature was a bright echogenic oval mass in association with the posterior mitral valve leaflet (Fig. 4). She had no clinical features suggestive of infective endocarditis, and it was thought that this mass was most probably a mucin deposit. The features of the mass were not in keeping with an atrial myxoma nor a vegetation. She has an annual echocardiography to assess the size of the mitral mass, which has so far remained unchanged, as has her mitral valve function. Treatment with frusemide 40 mg daily was initiated, which lessened her exertional dyspnoea but did not control her hypertension. Nifedipine 40 mg twice daily was given, although the hypertension was not ade- quately controlled and she complained of considerable 594 q 2001 British Association of Dermatologists