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In a recent issue of the Annals Hill et al reported a risk of cancer in patients with (systemic) scleroderma. The authors found an increased risk for all cancers, but the greatest significantly increased relative risk was for lung cancer.1
Published data do not indicate an increased risk of cancer for patients with localised scleroderma or morphoea in general.2 But that statement needs a closer look. Although there are no population based studies in this field, there is some evidence that patients with pansclerotic or generalised morphoea with longstanding ulcerations of skin or scars are at risk for the development of squamous cell carcinoma. In a recent overview we found seven case reports on squamous cell carcinoma in patients with pansclerotic or generalised morphoea.3 The number seems remarkably high because this disease is extremely rare. The observation has not only some impact on follow up but on treatment as well. The use of ultraviolet based treatments like UVA1 or PUVA (psoralen plus UVA) widely used successfully for more severe or widespread forms of morphoea4 might further increase the risk of squamous cell carcinoma in these patients.
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Author’s reply
Dr Wollina’s observations are of great clinical and research interest. Unfortunately, we excluded patients with localised scleroderma in our study1 owing to incomplete ascertainment in South Australia. We agree that further investigation of this interesting area is needed, particularly in relation to the influence of treatment. However, epidemiological studies of rare diseases are a challenging area of research.