Disabling Pansclerotic Morphea

Kim, Dong Seok; Pyun, Sae Hyun; Kim, Sang Won

Abstract

Disibling pansclerotic morphea, a severe form of generalized morphea, has a relentless clisabling course, producing marked contract,ion and joint deformity due to sclerosis of the skin and the deeper structures. We report herein an unusual case of morphea in a 25 year old male who had suffered from chronic recurrent ulceration with scar like sclerotic changes overlying limb folds, ankles, nape and abdomen since 4 years of age. The indurated lesions on the chest and abdomen were ivory colored, sclerotic guttae and plaques having a, violac ous border. At l4 years of age, the skin lesions were diagnostically ambiguous, wherea.s the immunologic results revealed a selective IgA deficiency with partial T lymphacyte inactivation. About 10 years later, the follow up visit showed this severe form of morphea over generalized area and disabling contractures of knees, elbows and alkles, but IgA value returned nearly to the nomal level.