Subcutaneous morphoea: a clinical study of sixteen cases

Sixteen patients had biopsy-proved inflammatory sclerosis of the panniculus or fascia, which usually was seen in a generalized distribution. Although the sclerosis involved the digits of five patients, it was the result of centrifugal spread of the sclerosis and was not accompanied by ulcers, calcinosis, distal phalangeal resorption or telangiectasia. Four patients may have had digital vasopasm in the extremities involved in the sclerotic process. Five patients also had cutaneous lesions of dermal morphoea or lichen sclerosus et atrophicus. Four patients had a reduced carbon monoxide diffusing capacity, and one of these had roentgenographic evidence of pulmonary fibrosis. In three patients, oesophageal motility or barium swallow studies showed evidence of sclerodermatous changes. The sclerotic process was a contributory cause of the death of one patient. Five patients had peripheral eosinophilia. Six patients seemed to have responded to anti-inflammatory agents. We believe that subcutaneous morphoea is generally more inflammatory than generalized morphoea of the dermal type and may be more likely to progress to mild systemic sclerosis. We suggest that response of subcutaneous morphoea to anti-inflammatory agents is simply a reflection of the degree of inflammation present.