Angiokeratomas in Juvenile Dermatomyositis

: Juvenile dermatomyositis (JDM) is an uncommon disease that features muscle weakness, a characteristic rash, and vascular changes in skin, muscle, and other organs. We report a patient with JDM who developed multiple angiokeratomas, one of which was calcified. Electron microscopy of an angiokeratoma revealed altered connective tissue consistent with abnormal collagen. To our knowledge, angiokeratomas occurring in association with JDM have not been previously reported. Abnormal collagen distinguishes the angiokeratomas in our patient from those seen in other conditions. We propose that the angiokeratomas resulted from a progressive compensatory response to the obliterative angiopathy of JDM.