An atypical case of scleroderma

Systemic sclerosis, or scleroderma is a rare systemic autoimmune rheumatic disease with an annual incidence of 3.7 per million in the UK. We report the case of a 55 year old female presenting with insidious onset weight loss, pruritus and worsening shortness of breath. Examination revealed signs of cardiac failure, fine inspiratory crepitations, hepatomegaly and diffuse cutaneous depigmentation although with minimal peripheral sclerodactyly. The anti-nuclear antibodies were interestingly negative and malignancy was not found on thorough screening. The patient was commenced on treatment with oral prednisone and intravenous cyclophosphamide for diffuse cutaneous systemic sclerosis. A rapid response in cardiac function, respiratory symptoms and fatigue was noted. In addition, skin texture rapidly improved with associated re-pigmentation seen within two weeks of commencing treatment. However, within two months of discharge the patient died. The cause of death was thought to be due to cardiac arrhythmia likely secondary to chronic myocarditis.