What diagnosis do these findings suggest?

The physical examination findings suggest mitral stenosis with atrial fibrillation.

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What is the underlying etiology?

Most cases of mitral stenosis are caused by chronic rheumatic heart disease, although more than 50% of these patients do not have a known history of rheumatic fever. In the acute phase, rheumatic fever may cause mitral regurgitation. Mitral stenosis may develop a few years later and symptoms may not develop until many years afterwards. The stenosis is due to the thickening of the valve leaflets with fibrous obliteration. There may be calcium deposition of the leaflets, chordae and the annulus with commissural and chordal fusion. Eventually, a funnel-shaped mitral valve with a fish-mouth orifice may occur.

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Echocardiogram is the most convenient and accurate investigation of choice. It provides a definitive diagnosis, it assesses the severity of the stenosis and it can also evaluate the suitability of the valve (by assessing the degree of calcification, thickening and mobility of the valve leaflets) for percutaneous balloon valvuloplasty.

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Patients without symptoms only require antibiotic prophylaxis for infective endocarditis. Those with mild symptoms may be treated with diuretics to lower the left atrial pressure. In patients with atrial fibrillation, rate control is important to increase diastolic filling time. Digitalis and beta-blockers are the drugs of choice. As patients with mitral stenosis and atrial fibrillation are prone to thromobembolism and stroke, anticoagulation therapy with warfarin is mandatory unless contraindicated. Attempts to restore sinus rhythm with antiarrhythmic drugs or cardioversion are likely futile unless the degree of mitral stenosis is minimal. For symptomatic patients with moderate or severe mitral stenosis, open heart surgery (open mitral valvotomy/ mitral valve replacement) or percutaneous balloon mitral valvuloplasty would be indicated. In general, if the mitral valve is pliable, mobile and not heavily calcified and there is no associated significant mitral regurgitation, valvuloplasty would be the first choice as it is a minimally invasive procedure compared with open heart surgery.

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What is the most likely clinical diagnosis?

The clinical diagnosis is chronic stage of lipodermatosclerosis. Lipodermatosclerosis is a progressive fibrotic process of the skin and subcutaneous fat, usually associated with chronic venous insufficiency. Another name is sclerosing panniculitis. In the acute stage, a poorly-defined cellulitis-like area of painful oedematous erythema develops usually on the medial calf near the ankle. With progression to the chronic stage, the affected area becomes progressively indurated, depressed and hyperpigmented. Ulceration may develop. There may be associated signs of chronic venous insufficiency. With time, the legs will look like an inverted bottle or bowling pin.

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What are the differential diagnoses?

In the acute stage, differential diagnoses include cellulitis, phlebitis, erythema nodosum, inflammatory morphea and other forms of panniculitis. The chronic stage of lipodermatosclerosis is usually clinically distinctive.

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What investigations will you perform for this lady?

A biopsy may be warranted during the acute stage to exclude other differential diagnoses, which will show septal and periseptal fibrosis with varying degree of sclerosis. The problem with biopsy is that the wound may not heal well especially for the chronic form of the condition. Workup of the venous system (e.g. duplex ultrasound) is useful to look for underlying cause of chronic venous insufficiency.

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How do you treat this lady?

Treatment is difficult. Treatment of underlying causes of venous insufficiency is warranted. Graduated compression stocking is helpful symptomatically for most patients and also helps in healing of venous ulcer. Oral medications like stanozolol and pentoxifylline may be tried.

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What is the prognosis?

Typical course of lipodermatosclerosis is chronic and progressive. Early recognition and treatment with compression and correction of underlying causes of venous insufficiency may prevent progression to ulcerative disease.

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