Unilateral linear pansclerotic morphea affecting face and limbs

Abstract

Disabling pansclerotic morphea is a rare atrophying and sclerosing disorder of the subcutaneous tissue, muscle and bone. It is characterized by atrophy of the skin, subcutaneous fat, muscle and bone involving half of the face. In some patients the atrophic lesions extend to involve the ipsilateral or contralateral upper and lower limbs with radiological evidence of hemiatrophy. The patients may present with arthralgia, convulsions or cramps. We report a case of a woman with deformity of face, and left upper and lower limbs that had started as an indurated plaque on the left half of forehead at the age of 5 years and had gradually enlarged, followed by the development of atrophic changes in left eye. The case is being reported in view of its rare occurrence.