Catalog

Disabling pansclerotic morphoea of childhood

No MMS code

Link to WHO Definition

Disabling pansclerotic morphoea of childhood – http://id.who.int/icd/entity/692165447

This is a rare, severe, mutilating form of morphoea involving the dermis, fat, fascia, muscle and even bone, usually starting before the age of 14 years. It may develop from linear morphoea. Superficial and deep cutaneous sclerosis involves the trunk and extremities, scalp and face, with sparing of the fingertips and toes. There may be a claw deformity of the hands and contracture of the Achilles tendons. Raynaud phenomenon is not found but oesophageal, pulmonary and periodontal changes may occur. Flexion contractures, osteoporosis and other bone changes are frequent.